THE  LIBRARY 

OF 

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OF  CALIFORNIA 

LOS  ANGELES 


7 


MEDICAL    OPHTHALMOLOGY 


U'o 

THE    MEMORY    OF 

WALTER  HAMILTON  HYLTON  JESSOP 

AND    TO 

WILLIAM  THOMAS  HOLMES  SPICER, 

MY  TEACHERS 
IN  AFFECTIONATE  REGARD. 


MEDICAL 
OPHTHALMOLOGY 


BY 

R.   FOSTER  MOORE,   O.B.E., 

M.A.,  B.Ch.  (Cantab.),  F.R.C.S. 

ASSISTANT    OPHTHALMIC   SURGEON,     ST.    BARTHOLOMEW'S   HOSPITAL. 
SURGEON,    MOORFIELDS    EYE   HOSPITAL. 


WITH  80  ILLUSTRATIONS. 


PHILADELPHIA 
P.   BLAKISTON'S   SON   &   CO. 

1012  WALNUT    STREET 
1922 


Printed  in  Great  Britain 


PREFACE 

IN  the  following  pages  an  attempt  has  been  made  to  give  a 
concise  account  of  those  pathological  conditions  of  the  eyes 
and  their  connections  which,  for  the  most  part,  are  of  interest 
in  general  medical  diseases. 

As  regards  my  personal  opinions  and  observations.,  they  are 
in  great  measure  the  results  of  investigations  carried  out  during 
the  last  nine  years  in  the  medical  wards  of  St.  Bartholomew's 
Hospital. 

The  changes  are  chiefly  considered  under  the  heads  of 
separate  diseases  ;  in  Chapter  I.,  however.  I  have  collected 
together  for  separate  consideration  a  heterogeneous  group  of 
important  ophthalmological  signs  and  symptoms  without 
special  reference  to  the  diseases  with  which  they  are  connected. 

The  subsequent  chapters  are  arranged  to  include  systemic 
groups,  e.g.,  "  Diseases  of  the  Nervous  System,"  '  Toxic 
Amblyopias,"  etc.  The  references  have  been  placed  at  the 
foot  of  each  page,  and  whilst  this  has  resulted  in  a  small  amount 
of  duplication,  it  was  felt  that  this  was  justified  by  the  readiness 
of  access  thus  provided. 

An  "  Index  of  Authors  "  has  been  added  in  addition  to  the 
general  Index. 

My  especial  thanks  are  due  to  Dr.  Hugh  Thursfield  and  Dr. 
John  Parkinson. 

Dr.  Thursfield  has  spent  much  time  and  trouble  in  reading 
the  proofs  through  for  me.  I  do  not  care  to  remember  the 
omissions,  inaccuracies  and  ambiguities  which  his  knowledge, 
and  skill  in  literary  matters  have  revealed  ;  all  his  suggestions 
have  been  adopted. 

Dr.  Parkinson,  in  the  earlier  stages,  has  made  many  valuable 
suggestions  from  his  wide  experience.  These  have  been  the 
means  of  saving  me  much  trouble  later.  He  has  also  given  me 
no  small  amount  of  practical  help. 


vi  PREFACE 

I  have  to  thank  my  colleagues  at  St.  Bartholomew's  and 
Moorfields  for  the  free  hand  which  they  have  given  me  with 
respect  to  the  patients  under  their  care.  This  has  made 
possible  many  of  the  observations. 

I  have  to  thank  Sir  John  Parsons  for  Fig.  77. 

Mr.  Humphrey  Neame,  Pathologist  at  Moorfields,  has 
prepared  some  of  the  specimens  for  me,  and  has  given  much 
help  in  their  reproduction.  I  have  to  thank  Mr.  Maurice 
Whiting  for  the  specimen  from  which  Fig.  26  is  made. 

Figs.  9,  18,  28,  37,  40,  48,  55  and  67  are  from  specimens 
belonging  to  Moorfields,  and  I  have  to  thank  my  colleagues 
there  for  permission  to  use  them. 

For  Fig.  74  I  am  indebted  to  the  British  Journal  of  Ophthal- 
mology ;  for  Figs.  14,  59,  60,  63,  72  and  73  to  Brain  ;  and  to 
the  Journal  of  Anatomy  and  Physiology  for  Figs.  1  and  2. 

Lastly,  I  have  to  thank  Messrs.  J.  and  A.  Churchill  for  the 
courtesy  with  which  they  have  endeavoured  throughout  to 
meet  my  wishes. 

R.   FOSTER  MOORE. 

HARLEY  STREET. 


TABLE    OF    CONTENTS 


CHAPTER    I 

The  cortical  representation  of  vision — Papilloedema — Nystagmus 
— Retinal  haemorrhages  in  general — The  hemianopic  pupil  re- 
action— Bi -nasal  hemianopia — The  retention  of  the  pupil 
reaction  in  blindness — The  indirect  paralysis  of  cranial  nerves 
— Monocular  diplopia — Haemorrhage  into  the  optic  nerve 
sheath — The  retina  at  death  ...... 


CHAPTER    II 

Arterio -sclerosis — Pernicious  anaemia — Leukaemia — Chloroma — 
Chlorosis — Secondary  anaemias — Purpura — Raynaud's  disease 
— Serious  loss  of  sight  from  loss  of  blood — Erythraemia 
(Vaquez's  disease) — Cyanosis  retinae — Retinal  haemorrhages 
in  new-born  infants — Thrombosis  of  retinal  veins — Throm- 
bosis of  the  cavernous  sinus  50 


CHAPTER    III 

Disturbances  of  visual  orientation — Lesions  of  the  sympathetic 
nerve — Hysteria — Epilepsy  —  Ophthalmoplegia  externa  — 
Ophthalmoplegia  interna  of  unknown  cause — Hydrocephalus 
—  Encephalitis  lethargica  —  Polio -encephalitis — The  intra- 
ocular lesions  in  tabes  and  general  paralysis — Friedreich's 
ataxia — Acute  myelitis  with  retrobulbar  neuritis — Dissemi- 
nate sclerosis — Syringomyelia — Myasthenia  gravis — Myotonia 
atropica  .  .  .......  93 


CHAPTER    IV 

Renal  disease — Gout — Diabetes — Grave's  disease — Disease  of  the 

pituitary  body — Infantile  scurvy — Keratomalacia         .          .      131 

CHAPTER   V 

Syphilis — Gross     tuberculous     lesions  —  Miliary     tuberculosis  — 
Cerebro -spinal  meningitis — Pyaemia  and  septicaemia — Inf  c- 
tive  endocarditis — Facial  erysipelas — Gonococcal  eye  affec- 
tions— Eye  affections  in  dysentery — Tetanus — Diphtheria —  . 
Leprosy  ........••      190 


viii  TABLE  OF  CONTENTS 

CHAPTER   VI 

Leber's  disease — Amaurotic  family  idiocy  (Tay-Sach's  disease) — 
Congenital  word  blindness — Night  blindness — Blue  sclerotics 
— Ocular  torticollis — Jaw  blinking — Cyclic  dilatation  of  the 
pupil — Abnormalities  of  the  retinal  vessels — Medullated 
nerve  fibres  .  .  .  .  .  .  .214 


CHAPTER   VII 

Oxycephaly — Leontiasis  ossea — Migraine — Recurrent  intra-ocular 
haemorrhage  (Bales'  disease)  —  Herpes  ophthalmicus  — 
Mikulicz's  syndrome — Lactation — Mongolian  idiocy — Acute 
amaurosis  of  infants  234 


CHAPTER   VIII 

Snow    blindness  —  Eclipse    blindness  —  Electric    amblyopia  — 
Lightning— Neuroparalytic  keratitis  —  Retro-bulbar  neuritis 
—Oyster  shucker's  keratitis       .          .          .          .          .          .251 


CHAPTER    IX 

Tobacco — Alcohol— Methyl  alcohol — Quinine — Sodium  salicylate 
—  Lead  —  Silver  nitrate  —  lodof orm  —  Carbon  di -sulphide — 
Nitro-benzol — Aniline — Phenol — Ochronosis — Naphthaline — 
Antipyrin — Santonin — Aspidium  (Filix  Mas) — The  arylar- 
senates — ^Ethylhydrocuprein  (Optochin)  .  .  .  .258 


CHAPTER   X 

Cysticercus  —  Echinococcus — Trypanosomiasis — Malaria — Filaria 
loa — Sporotrichosis — Actinomycosis    .... 


INDEXES 


281 


287 


MEDICAL  OPHTHALMOLOGY 


CHAPTER   I 

The  cortical  representation  of  vision— Papilloedema — Nystagmus — 
Ketinal  haemorrhages  in  general — The  hemianopic  pupil  reaction — 
Bi -nasal  hemiauopia — -The  retention  of  the  pupil  reaction  in 
blindness — The  indirect  paralysis  of  cranial  nerves — Monocular 
diplopia — Haemorrhage  into  the  optic  nerve  sheath — The  retina 
at  death. 

THE   CORTICAL   REPRESENTATION   OF   VISION. 

IT  has  long  been  recognised  that  the  visual  cortical  area  of 
the  brain  is,  for  the  most  part,  situated  on  the  inner  aspect  of 
the  occipital  lobe. 

It  is  probable  that  the  area  striata  of  the  brain,  i.e.,  that  part 
of  the  cerebral  cortex  in  which  the  stria  of  Gennari  occurs, 
represents  the  true  visual  cortex,  and  Elliot  Smith  l  has  shown 
how  exceedingly  sharply  defined  is  this  area,  and  how  easily 
this,  as  well  as  other  portions  of  the  cortex,  may  be  delimited 
by  macroscopic"methods. 

The  limits  of  the  area  are  thus  defined  by  him  : — 

"  Not  only  are  both  walls  of  the  sulcus  calcarinus  (posterior 
calcarine  fissure,  Cunningham)  formed  entirely  of  area  striata, 
but  part  of  the  lingual  convolution  below  it  is  also  formed  of 
area  striata  "  (Fig.  1,  p.  2). 

The  dorsal  boundary  of  the  area  striata  in  the  cuneus  is  the 
sulcus  cunei,  which  Elliot  Smith  has  named  the  Sulcus  Limitans 
Superior  Area  Striatae. 

The  ventral  boundary  of  the  area  striatse  in  the  lingual  gyrus 
is  the  sulcus  lingualis  which  thus  becomes  the  Sulcus  Limitans 
Inferior  Arese  Striatae. 

1  Jour.  Anat<  and  Physiol.,  1907,  xli.,  200. 


2  CORTICAL  REPRESENTATION 

"  If  these  two  sulci,  the  sulcus  cunei  and  the  sulcus  lingualis, 
which  limit  the  area  striata  dorsally  and  ventrally  respectively 
to  the  posterior  calcarine  fissure,  Be  traced  backwards,  it  will 
be  found  in  many  cases  that  these  fissures  each  form  a  semi- 
circular sulcus  which  has  been  termed  polar  (Fig.  2,  p.  3), 
and  which  may  be  named  the  Sulci  Polaris  Superior  and 


I.OCC.»»*M. 


*.c«ie    - 
Area  striata   -' 

S.IM.INT.  4B.ITB.    - 


s.  pc 


FIG.  1.- — A  diagram  to  represent  the  distribution  of  the  different  cortical 
areas  in  a  coronal  section  through  the  left  hemisphere  one  centimetre 
behind  the  fossa  parieto-occipitalis.  LV.  =  Posterior  cornu  of  lateral 
ventricle,  s.  calc.  =  posterior  calcarine  sulcus.  s.  Urn.  sup.  ar.  sir.  = 
sulcus  cunei  (sulcus  limitans  superior  area?  striatae).  .9.  Urn.  inf.  ar. 
xlr.  =  sulcus  lingualis  (sulcus  limitans  inferior  areae  striatse).  Note 
how  the  stripe  of  Gennari  (the  thick  black  line)  ends  abruptly  at  the 
bottom  of  the  sulcus  cunei  and  the  sulcus  lingualis.  (After  Elliot 
Smith.) 

Inferior.  The  area  striata  extends  as  far  as  the  lip  of  these 
furrows  without  entering  into  the  formation  of  their  walls  ;  in 
the  case  of  the  cuneal  and  lingual  sulci  the  area  striata  extends 
into  the  furrow  and  stops  in  its  floor  (Fig.  1).  The  area  striata 
usually  extends  on  to  the  lateral  surface  of  the  hemisphere  and 
is  folded  axially  to  form  the  sulcus  calcarinus  externus  of 
Cunningham,  and  ends  at,  or  close  to,  the  caudal  lip  of  the 
sulcus  lunatus.  The  area  striata  forms  the  ventral  wall  only 


AREA   8TRIATA  3 

of  the  anterior  calcarine  fissure  (Cunningham)  and  stops  in  the 
floor  of  the  sulcus  (Fig.  2).  When  the  gyrus  cunei  is  reached, 
the  area  striata  crosses  the  floor  of  the  furrow  and  extends 
exactly  as  far  as  the  crest  of  the  small  gyrus." 

These,  then,  are  the  topographical  boundaries  of  that  part 
of  the  cerebral  cortex  in  which  the  stria  of  Gennari  is  present, 
to  which  the  name  area  striata  has  been  applied,  and  which  is 


s.  polaris.  sup. 


V5°P  rfe»«*»l 

@JJZ®M PRAEOUHEUS 

sfe&\ 


NY*  •>•**• 

•v^i±5. 


coap  C/M.I 


»«, 


S-  polaris  inf. 


\ 


TEMPORAL 
AREA 


FIG.  2. — A  diagram  to  illustrate  the  distribution  of  the  cortical  areas  on 
the  mesial  surface  of  the  occipital  region  of  the  left  hemisphere.  The 
two  arrows  indicate  the  plane  of  the  section  shown  in  figure.  The 
area  striata  is  represented  by  dots  ;  the  area  parastriata  by  crosses  ; 
the  area  peristriata  by  circles.  (After  Elliot  Smith.)  Compare  the 
above  with  Gordon  Holmes'  diagram  (Fig.  3,  p.  4),  arrived  at  on  clinical 
grounds,  and  with  the  cortical  visual  area  as  defined  by  Schafer 
(p.  7),  and  arrived  at  as  a  result  of  physiological  investigation. 

believed  to  represent  the  part  of  the  cortex  which  is  concerned 
with  vision. 

From  the  clinical  side  our  knowledge  has  been  advanced  and 
placed  on  a  secure  basis  as  a  result  of  the  careful  and  thorough 
work  of  a  number  of  observers,  especially  of  Gordon  Holmes,  and 
Gordon  Holmes  and  Sir  William  Lister. 

1—2 


4  CORTICAL   REPRESENTATION 

These  investigators  1  examined  thoroughly  a  large  number 
of  men  who  were  wounded  in  the  occipital  region  of  the  skull, 
and  correlated  the  position  of  the  injury  to  the  brain  with  the 
visual  fields,  as  taken  either  with  a  perimeter  or  by  means  of  a 


Fiu.  IJ. — A  diagram  of  the  probable  representation  of  the  different  portions 
of  the  visual  fields  in  the  calcarine  cortex.  Fig.  3  is  a  drawing  of  the 
mesial  surface  of  the  left  occipital  lobe  with  the  lips  of  the  calcarine 
fissure  separated  so  that  its  walls  and  floor  are  visible.  The  markings 
'of  the  various  portions  of  the  visual  cortex  which  is  thus  exposed 
correspond  with  those  shown  on  the  chart  (Fig.  4)  of  the  right  half 
»f  the  field  of  vision.  (Gordon  Holmes.) 

Bjerrum's  screen.  The  site  of  the  damage  to  the  brain  was  in 
some  cases  confirmed  at  autopsy,  in  other  cases  it  was  judged 
either  at  operation,  or  arrived  at  by  craniometric  measure- 
ments. 

1  Brit.  Jour,  of  Ophth.,  1918,  353  ;  Proc,  Royal  Soc,  of  Med.,  Section  of  Ophth., 
1916,  ix.,  57. 


OPTIC  RADIATIONS 


A  complication  which  gave  rise  to  some  difficulty  in  correlating 
the  site  of  the  wound,  with  the  resulting  defects  of  the  visual 
fields,  was  the  fact  that  a  projectile  which  had  damaged  the 
cerebral  cortex  had  often  traversed  a  part  of  the  brain,  and  in 


FIG.  4. 

so  doing  had  damaged  the  fibres  of  the  optic  radiations  as  well 
as  the  cortical  area. 

On  this  account  it  was  difficult  to  apportion  accurately,  the 
defect  which  was  due  to  damage  suffered  by  fibres  coming 
from  the  mid  brain,  and  that  which  was  due  to  cortical 
destruction. 

Central   Vision. — The   part   of   the   cortex   concerned   with 


6  CORTICAL  REPRESENTATION 

central  vision,  i.e.,  macular Representation,  is  at  the  occipital 
poles,  extending  on  to  the  lateral  surface  of  the  brain,  for 
number  of  cases  have  been  observed,  in  which  damage  limited 
to  one  or  to  both  occipital  poles  produced  respectively,  an 
homonymous  paracentral  scotoma,  or  a  central  scotoma  in 
both  eyes,  the  peripheral  fields  being  complete. 


255 


FIG.  5. 

FIGS.  5  and  6  are  the  visual  fields  from  a  man  of  sixty-three  who  had 
symptoms  of  thrombosis  of  the  right  posterior  cerebral  artery. 

In  the  unilateral  injuries  in  which  an  homonymous  para- 
central  scotoma  was  present,  this  always  extended  right  up  to 
the  fixation  point,  and  Wilfred  Harris  *  has  observed,  that  in 
the  temporary  hemianopia  which  may  accompany  migraine, 
the  dividing  line  between  the  seeing  and  blind  halves  also  passes 
through  the  fixation  point. 

It  has  for  long  been  recognised  that  in  cases  of  homonympus 
hemianopia  in  civil  practice  due  to  obstruction  of  the  posterior 
cerebral  artery,  whether  by  an  embolus  or  as  a  result  of  throm- 

1  Brain,  1897,  xx.,  360. 


THE  MACULA  7 

bosis,  the  scotoma  falls  short  of  the  fixation  point  by  about 
10  degrees  (Fig.  5  and  6),  and  this  has  given  rise  to  the  sug- 
gestion that  each  macula  is  represented  in  each  cerebral  cortex 
right  and  left. 

In  view  of  the  before-mentioned  facts  it  is  clear  that  this 
explanation  can  no  longer  be  maintained,  and  it  is  suggested 
by  Holmes  and  Lister  that  the  explanation  of  the  escape  of  the 
macula  in  vascular  lesions  is  to  be  found  in  the  fact,  that  the 


285 


FIG.  6. 


occipital  pole  is  a  borderland  territory  as  regards  the  distribu- 
tion of  the  posterior  and  middle  cerebral  arteries,  and  that  if 
one  of  those  vessels  is  blocked  the  other  will  supply  sufficient 
blood  for  the  needs  of  this  area  of  the  cortex. 

With  regard  to  the  representation  of  the  other  parts  of  the 
retina  Schafer  *•  gives  them  as  the  result  of  experimental  work 
as  follows : — 

(1)  The  whole  of  the  visual  area  of  one  hemisphere  is 
1  "  Text  Book  of  Phys.,"  Edinburgh  and  London,  1900,  ii.,  757. 


8  CORTICAL  REPRESENTATION 

connected    with    the    corresponding    lateral     half     of     both 

retinae. 

(2)  The  upper  zone  of  the  visual  area  of  one  hemisphere  is 
connected  with  the  upper  zone  of  the  corresponding  lateral  half 
of  both  retinae. 

(3)  The  lower  zone  of  the  visual  area  of  one  hemisphere  is 


FIG.  7. 

FIGS.  7  and  8  represent  a  nearly  complete  inferior  hemianopia  from  a 
gun-shot  injury  to  the  occipital  cortex  and  optic  radiations.  The 
wound  of  entry  was  1  inch  behind  the  left  parietal  eminence,  the 
exit  wound  being  1 J  inches  to  the  right  of  the  middle  line  and  about 
1J  inches  above  the  external  occipital  protuberance.  Central  visual 
acuity  was  unimpaired.  The  fields  were  taken  five  years  after  the 
injury. 

connected  with  the  lower  zone  of  the  corresponding  lateral  half 
of  both  retinae. 

(4)  The  intermediate  zone  of  the  visual  area  is  connected  with 
the  middle  zone  of  the  corresponding  lateral  half  of  both  retinae. 

Holmes  and  Lister's  results  are  in  large  measure  in  accord 
with  the  foregoing. 

They  found  that  wounds  which  involve  the  upper  lip  of  the 


posterior  calcarine  fissure  produce  a  corresponding  homony- 
mous  loss  in  the  lower  visual  fields,  showing  that  the  upper  parts 
of  the  retina  are  represented  in  the  upper  lips  of  the  fissure. 

The  charts  (Fig.  7  and  8)  were  obtained  from  a  soldier  about 
five  years  after  being  wounded  by  a  rifle  bullet :  the  entrance 
wound  was  about  1  inch  behind  the  left  parietal  eminence,  the 
exit  was  1|  inches  to  the  right  of  the  middle  line  and  1^  inches 
above  the  external  occipital  protuberance ;  his  central  vision 
was  not  affected.  It  seemed  probable  that  the  damage  on  the 


left  side  of  the  brain  was  chiefly  to  the  optic  radiations,  and  on 
the  right  side  the  cortex  was  destroyed. 

It  is  remarked  by  Holmes  that  cases  which  demonstrate  a 
loss  of  the  upper  field  of  vision  with  damage  which  could,  with 
good  reason,  be  referred  to  the  lower  lips  of  the  calcarine  fissure 
are  much  less  common,  yet  all  observations  go  to  show  that  this 
relationship  does  exist. 

It  seems  clear  that  the  upper  parts  of  the  retina  are  repre- 
sented in  general  along  the  upper  margin  of  the  posterior 


10  CORTICAL  REPRESENTATION 

calcarine  fissure,  and  the  lower  parts  along  the  lower  lips  of  this 

fissure. 

/  Further,  lesions  which  had  damaged  the  more  anterior  parts 

of  this  region,  i.e.,  at  the  apex  of  the  cuneus,  gave  rise  to  defects 

at  the  periphery  of  the  field  of  vision  (Figs.  3  and  4),  and  suggest, 

therefore,  that  the  more  peripheral  parts   of  the  retina  are 

represented  far  forwardsy 

It  will  be  clear  that  few  cases  can  occur  where  the  cortex  at 
the  bottom  of  the  calcarine  fissure  is  damaged  without  compli- 
cating damage  to  surrounding  cortex  or  optic  radiations. 

A  case  of  Wilbrand  and  Henschen's,  quoted  by  Holmes,  in 
which  a  narrow  homonymous  scotoma  extended  from  the 
fixation  point  horizontally  outwards  to  60  degrees,  and  in  which 
it  was  found  that  softening  of  the  brain  was  present  at  the 
bottom  of  the  calcarine  fissure,  suggests  that  the  horizontal 
parts  of  the  retina  are  represented  along  the  deeper  parts  of  the 
area  striata,  and  Holmes  states  that  he  has  seen  many  cases 
where  superficial  injuries  of  the  mesial  aspect  of  one  hemisphere 
were  associated  with  blindness  along  the  vertical  meridians  of 
the  visual  fields. 

Lesions  of  the  Optic  Radiations. — I  have  seen  a  man  in  whom 
a  shrapnel  ball  had  entered  over  the  left  ear  and  had  come  to 
rest  on  the  tentorium  cerebelli  of  the  left  side.  It  seemed  that 
it  must  have  been  the  lower  fibres  of  the  radiations  which  were 
damaged  and  that  injury  to  the  cortex  could  be  excluded ;  he 
had  a  sharp  cut  right  superior  quadrantic  hemianopia. 

Holmes  has  seen  cases  where  it  was  judged  that  the  damage 
involved  the  ('more  dorsal  fibres  of  the  radiations,  and  which 
were  associated  with  inferior  quadrantic  hemianopia)  He 
states  "  it  seems  tempting  to  assume,  that  even  in  the  main 
mass  of  the  radiations,  those  indirectly  connected  with  the 
upper  and  lower  halves  of  the  retina  are  contained  in  distinct 
bundles  separated  from  each  other  by  an  anatomical  interval, 
but  this  hypothesis  seems  a  priori  so  improbable,  that  it  cannot 
be  accepted  without  further  evidence,  and  unless  an  anatomical 
basis  for  it  can  be  found." 

The  Nature  of  the  Brain  Lesions. — In  many  cases  of  gun-shot 
wound  of  the  brain  it  will  be  found  that  in  the  early  days  after 


RECOVERY  OF  FIELD  11 

i  ^» 

the  injury  marked  recovery  of  vision  occurs,  and  according  to 
Hine  x  the  recovery  starts  with  central  vision  and  extends 
peripheral  wards  from  here,  but  in  other  cases  the  reverse 
sequence  is  seen. 

It  would  seem  that  in  these  cases  the  temporary  visual  loss  is 
due  to  concussion  in  the  surrounding  brain,  and  that  there  is  no 
constant  rule  as  to  whether  recovery  takes  place  from  the 
periphery  towards  the  macula  or  vice  versa. 

The  sequence  of  recovery  probably  depends  entirely  upon  the 
position  of  the  damage  done,  and  takes  place  from  the  less 
damaged  towards  the  more  damaged  area,  until  the  limit  of 
those  parts  which  are  permanently  destroyed  is  reached,  where 
no  recovery  can  occur  and  a  corresponding  permanent  defect 
remains.  In  cases  which  were  examined  three  weeks  or  more 
after  the  receipt  of  the  injury  the  edges  of  the  scotoma  were 
usually  quite  sharp  and  did  not  change  afterwards. 

Riddoch  2  believes  that  in  an  area  of  the  visual  field  in  which 
recovery  is  taking  place,  light  and  movement  are  the  first 
stimuli  to  be  appreciated,  and  that  consequently  the  investiga- 
tion of  the  field  for  movement  is  of  considerable  prognostic 
importance. 

Losses  of  the  visual  fields  for  colour  have  frequently  occurred, 
but  never,  in  Holmes'  experience,  apart  from  constriction  of  the 
fields  for  white;  he  has  seen  no  case  in  which  there  was  a 
perfectly  intact  field  for  white  but  loss  of  the  fields  for  colours. 

Subjective  Visual  Phenomena. — It  is  not  uncommon  for 
subjective  visual  phenomena  to  occur  during  the  first  few  weeks 
after  the  receipt  of  an  injury  to  the  visual  cortex,  but  they  are 
much  less  common  in  the  later  periods. 

These  phenomena  at  times  take  the  form  of  coloured  lights 
and  flashes,  thus  suggesting  that  colour  perception  is  resident 
in  the  calcarine  region  of  the  cortex. 

The  following  are  Gordon  Holmes'  conclusions  (Figs.  3 
and  4)  : — 

(1)  The  upper  half  of  each  retina  is  represented  in  the  dorsal, 
and  the  lower  in  the  ventral,  part  of  each  visual  area. 

1  Brit.  Jour,  of  Ophth.,  1918,  ii.,  12. 

2  Brain,  1917,  xl.,  15. 


12  PAPILL(EDEMA 

(2)  The  centre  for  macular  or  central  vision  lies  in  the  most 

posterior  part  of  the  visual  cortex,  probably  on  the 
margins  and  on  the  lateral  surfaces  of  the  occipital 
poles.  The  macula  has  not  a  bilateral  representation. 

(3)  The  centre  for  vision  subserved  by  the  periphery  of  the 

retina  is  situated  in  the  anterior  portions  of  the  visual 
areas,  and  the  serial  concentric  zones  of  the  retina  from 
the  macula  to  the  periphery,  are  probably  represented 
in  this  order  from  behind  forwards  in  the  visual  cortex. 

(4)  Those  portions  of  the  retina  adjoining  the  vertical  axes 

are  probably  represented  in  the  dorsal  and  ventral 
margins  of  the  visual  areas,  whilst  the  retina  in  the 
neighbourhood  of  its  horizontal  axis  is  projected  on  the 
walls  and  the  floor  of  the  calcarine  fissures. 

(5)  Given  lesions   of  the  visual   cortex   produce   complete 

blindness  in  the  corresponding  portions  of  the  visual 
fields,  or  if  incomplete  an  amblyopia,  colour  vision  being 
generally  lost  and  white  objects  being  indistinct,  or 
only  more  potent  stimuli  as  abruptly  moving  objects, 
may  excite  sensations. 

(6)  The  defects  of  vision  in  the  fields  of  the  two  eyes  are 

always  congruous  and  superimposable,  provided  that 
no  abnormality  of  the  peripheral  visual  apparatus  exists. 

(7)  Lesions    of    the    lateral    surfaces    of    the    hemispheres, 

particularly  of  the  posterior  parietal  regions,  may  cause 
certain  disturbances  of  the  higher  visual  perceptual 
functions  with  intact  visual  sensibility,  as  loss  of  visual 
orientation  and  localisation  in  space,  disturbance  of  the 
perception  of  depth  and  distance,  visual  attention  loss, 
and  visual  agnosia. 

PAPILLCEDEMA. 

This  term,  first  introduced  by  Parsons  to  indicate  the  patho- 
logy of  the  condition,  has,  in  large  measure,  replaced  such  terms 
as  optic  neuritis,  papillitis,  choked  disc,  etc. ;  and  there  seems  no 
doubt  that  in  the  most  important  group  of  cases,  where  the 
disc  changes  are  an  indication  of  increased  intra-cranial  pressure, 


PATHOLOGY  13 

they  are  due  to  an  oedema  of  the  nerve  head  which  has  a  physical 
basis. 

Pathology. — The  pathology  of  the  condition  has  been  worked 
out  with  especial  care  by  Parsons,  and  Paton  and  Gordon 
Holmes.1 

They  show  that  the  swelling  of  the  disc  is  due  almost  entirely 
to  infiltration  with  fluid  of  its  tissues  and  the  anterior  layers  of 
the  lamina  cribrosa.  As  the  disc  swells  it  displaces  the  retina 


* 


FIG.  9. — A  longitudinal  section  through  the  optic  disc  in  a  case  of  papill- 
cedema,  showing  its  prominence  due  to  cedematous  swelling. 

laterally  (Fig.  9),  often  throwing  it  up  into  small  folds,  which 
may  be  seen  with  the  ophthalmoscope  as  faint  lines  concentric 
with  the  disc  margin.  As  the  swelling  increases  the  even  course 
of  the  nerve  fibres  becomes  disturbed,  and  some  of  the  more 
peripheral  ones  take  on  a  somewhat  acute  S-shaped  curve. 
The  axial  fibres  become  raised  to  fill  up  the  physiological  cup, 
which  is  further  filled  in  by  the  collection  of  oedema  fluid  under 
the  internal  limiting  membrane. 

The  alterations  in  the  other  layers  of  the  retina  are  surpris- 

1  Brain,  1911,  xxxiii.,  389. 


14  PAPILLCEDEMA 

ingly  slight,  and  are  limited  to  a  little  oedema  in  the  immediate 
proximity  of  the  disc. 

Changes  in  the  Vessels. — There  is  well-marked  distension  of 
the  veins  and  capillaries,  and  haemorrhages  on  the  disc  and  in 
its  immediate  vicinity  are  usually  present  in  a  case  of  any 
severity. 

Inflammation. — With  regard  to  the  presence  of  histological 
evidence  of  inflammation,  Paton  and  Holmes  found  slight  small- 
celled  infiltration  into  the  connective  tissue  sheath  of  some  of 
the  vessels  in  cases  where  papillcedema  had  been  intense,  it  was, 
however,  so  infrequent,  so  scanty,  and  involved  so  few  of  the 
vessels — most  of  them  being  quite  free — that  they  attribute  it 
to  a  reaction  of  the  tissues  to  degenerative  and  destructive 
processes  which  are  going  on  in  the  nerve. 

They  state  further,  that  the  histological  characters  of  papill- 
cedema, no  matter  at  what  stage  of  its  development  they  may 
be  examined,  are  not  those  of  an  inflammatory  process,  and  that 
in  two  cases  in  which  papilloedema  occurred  in  septic  menin- 
gitis, in  which  the  meningeal  inflammation  extended  to  the 
optic  sheath,  there  was  as  little  evidence  of  inflammatory 
changes  in  the  papilla  as  in  cases  due  to  cerebral  tumour. 

When  the  condition  has  been  in  progress  for  some  time, 
degenerative  processes  occur  which  are  manifested  by  the 
nerve  fibres  becoming  irregularly  swollen  and  varicose,  and 
later  breaking  up  into  degenerative  masses  with  nucleus-like 
centres — the  so-called  cytoid  bodies.  These  in  course  of  time 
undergo  lipoid  degeneration,  as  shown  by  Parsons,  and  ulti- 
mately disappear  ;  the  ganglion  cells  become  shrunken  and 
diminished  in  number. 

Sclerotic  Changes. — As  the  papillcedema  subsides,  if  secondary 
atrophy  results,  conspicuous  histological  changes  coincidentally 
appear.  The  disc  shrinks,  there  is  a  diminution  of  the  nerve 
fibres,  a  proliferation  of  the  neurogliai~nuclei  and  fibres,  and  an 
increase  of  the  fibrous  tissue  which  surrounds  the  vessels. 

In  a  case  in  which,  as  a  result  of  a  decompression  operation, 
subsidence  of  the  papillcedema  occurs  without  consecutive 
atrophy,  there  is  very  little,  if  any,  neuroglial  increase. 

Relation  of  the  Ophthalmoscopic  Appearances  to  the  Histo- 


PATHOGENESIS 


15 


OPTIt.    HCKff. 


logical  Changes.  —  The  blurring  and  obscuration  of  the  disc 
edges  is  partly  due  ^6  the  increased  depth  of  the  tissues  forming 
the  nerve  head,  and  partly  to  the  dispersal  of  light  by  the 
interspersed  fluid  (Fig.  9). 

The  greyish-white  striation  of  the  disc  and  adjoining  retina 
is  due  to  the  separation  of  the  nerve  fibres  by  oedema. 

Hemorrhages  are  either  flame-shaped,  if  in  the  nerve  fibre 
layer,  more  solid  and  homogeneous  if  between  the  internal 
limiting  membrane  and  the  nerve  fibres,  or  more  or  less  circular 
if  in  the  deeper  parts  of  the 
retina. 

The  soft-edged  white  patches 
near  the  disc  are  produced  by 
the  swelling  of  the  nerve  fibres 
and  the  products  of  their 
degeneration,  varicose  nerve 
fibres  and  cytoid  bodies. 

The  opaque  white  appear- 
ance of  the  atrophic  disc  is 
produced  by  the  occlusion  of 
the  smaller  vessels  and  the 
glial  sclerosis,  the  white  lines 
along  the  veins  on  the  disc  are 
due  to  thickening  of  their 
walls,  and  the  diminution  in 
the  size  of  the  arteries  is  due  to  their  compression  in  the 
sclerosed  tissues. 

Pathogenesis  of  Papilloedema. — It  has  been  shown,  that  from 
a  histological  point  of  view,  the  changes  in  the  nerve  head  are 
those  of  a  simple  oedema,  and  the  most  complete  and  satis- 
factory explanation  of  its  mode  of  production  is  that  of  a 
physically  produced  oedema,  due  to  obstruction  to  the  outflow 
of  blood  and  lymph  from  the  eye. 

Let  it  be  borne  in  mind  that  the  subarachnoid  space  of  the 
vaginal  sheath  of  the  optic  nerve  is  in  free  and  direct  communi- 
cation with  the  cerebral  subarachnoid  space  (vide  p.  47),  so  that 
any  increase  in  the  intra-cranial  pressure  is  transmitted  directly 
to  this  space. 


FIG.  10. 


16  PAPILUEDEMA 

If  now  the  central  retinal  vessels  be  traced  backwards  from 
the  retina,  they  lie  at  first  in  the  substance  of  the  optic  nerve, 
but  about  12  mm.  behind  the  globe  they  leave  the  nerve  and 
traverse  this  subarachnoid  space  in  order  to  pierce  the  dura, 
and  so  reach  their  parent  trunks  in  the  orbit  (Fig.  10). 

As  they  traverse  the  space,  artery,  vein,  and  lymphatics  will 
be  subject  to  any  increase  of  pressure  that  may  exist,  but 
whereas  the  artery  contains  blood  at  a  pressure  which  is 
estimated  at  100  mm.  Hg.  by  Leonard  Hill,  or  80  to  100  by 
Duverger  and  Barre,1  the  fluid  in  the  vein  and  lymphatics  is 
at  a  much  lower  pressure,  consequently  the  inflow  of  blood  to 
the  eye  will  be  but  little  affected,  but  the  outflow  of  blood  and 
of  lymph  from  the  nerve  head  into  the  sheath  space  will  be 
obstructed  ;  there  will  be  as  a  consequence  increased  transu- 
dation  of  lymph,  with  oedema  of  the  nerve  head. 

This  method  of  production  of  papilloadema  supposes  no 
interference  with  the  choroidal  circulation,  and  no  changes  in 
this  membrane  are  found. 

Confirmation  of  this  theory  is  supplied  by  the  fact,  that  in 
the  case  of  a  tumour  which  presses  directly  on  the  nerve  at 
the  optic  foramen,  the  subarachnoid  space  of  the  nerve  is  shut 
off  from  the  cerebral  subarachnoid  space,  and  so  the  retinal 
vessels  as  they  traverse  this  space,  are  protected  against  any 
increase  of  intra-cranial  pressure  that  may  occur. 

In  such  a  case,  then,  papillcedema  should  not  develop. 

This  is  exemplified  in  pituitary  tumours,  which  most  often 
produce  a  primary  optic  atrophy  from  pressure  upon  the 
nerve,  and  sometimes  a  frontal  tumour  may  press  directly 
upon  one  optic  nerve,  producing  primary  atrophy  in  it,  whilst 
papillosdema  is  developed  in  the  other  eye,  its  subarachnoid 
space  remaining  in  free  communication  with  the  subarachnoid 
space  of  the  brain,  and  so  its  retinal  vessels  are  not  protected 
against  the  increased  intra-cranial  pressure. 

It  may,  however,  be  remembered,  that  pressure  on  the  nerve 
at  the  optic  foramen  which  is  sufficient  to  produce  optic  atrophy 
will  probably  also  obstruct  the  ophthalmic  artery,  and  so  will 
diminish  the  inflow  in  the  retinal  artery. 

1  Arch.  cTOphth.,  1920,  xxxvi.,  71. 


OPHTHALMOSCOPIC  CHANGES  17 

Papilloedema  and  other  Disc  Changes. — Papillcedema  is  seen 
in  its  most  characteristic  form  in  cases  of  cerebral  tumour,  in 
some  cases  of  intra -cranial  abscess,  meningitis,  and  renal 
disease. 

There  are,  besides,  a  number  of  conditions,  such  as  retro- 
bulbar  neuritis,  disseminate  sclerosis,  some  cases  of  meningitis, 
arterio -sclerosis,  etc.,  in  which  changes  occur  in  the  disc  which 
are  limited  to  haziness  of  its  edge,  slight  swelling  and  a  few 
haemorrhages,  but  without,  as  a  rule,  any  high  degree  of  oedema 
or  swelling. 

These  changes  are,  as  a  rule,  included  under  the  term  papill- 
cedema,  although  they  differ  in  appearance  and  in  clinical 
significance  from  those  which  occur  in  their  typical  form  in 
cases  of  cerebral  neoplasm.  Thus,  at  the  present  time,  the 
mild  ophthalmoscopic  changes  which  may  occur,  for  example, 
in  the  nerve  head  from  the  presence  of  a  focus  of  degeneration 
close  behind  the  globe  in  a  case  of  disseminate  sclerosis,  are 
not  differentiated  by  name  from  the  high  degree  of  swelling 
and  oedema  of  the  disc  which  results  from  the  presence  of  a 
cerebral  tumour,  and  which  is  probably  due  immediately  to 
a  heightened  intra-cranial  pressure. 

Some  German  writers,  such  as  Uhthoff,  do,  however,  draw  a 
rather  artificial  distinction,  in  that  those  cases  in  which  swelling 
is  less  than  2  D.  are  called  optic  neuritis,  whilst  those  with  more 
than  this  degree  of  swelling  are  termed  choked  disc. 

Ophthalmoscopic  Appearances. — Without  describing  the 
changes  as  they  are  seen  with  the  ophthalmoscope,  it  may  be 
said  that  the  haziness  of  the  disc  margins  most  often  begins 
in  the  upper  nasal  quadrant,  that  the  appearance  of  the  disc 
may  remain  unchanged  for  a  long  period — many  months  or 
£ven  a  year  if  a  palliative  operation  is  not  performed — and  that 
at  times  small  haemorrhages  on  the  disc  may  precede  any  other 
sign  of  papilloedema  by  as  long  as  several  weeks.  In  some  cases, 
especially  in  children,  a  series  of  fine,  white  dots  is  developed 
in  lines  radiating  from  the  yellow  spot,  simulating  the  condi- 
tion often  seen  in  renal  retinitis  ;  they  differ,  however,  from 
the  renal  changes  in  that  the  dots  are  finer,  and  are  usually 
limited  to  the  side  of  the  yellow  spot  towards  the  optic 


18  PAPILLCEDEMA 

disc,  i.e.,  they  form  a  fan-shaped  figure  between  the  disc  and 

macula,  rather  than  a  star-shaped  figure  which  surrounds  the 

macula. 

Incidence.  —  Uhthoff  l  gives  the  following  table  of  the  causes 

of  papillcedema  :— 

Per  cent. 
Brain  tumour          .          .          .          .          .71 

Brain  tuberculoma  .          .          .          .3-6 

Cerebral  syphilis     .          .          .          .          .12 

Oxycephaly  .          .          .          .          .          .2-2 

Brain  abscess          .          .          .          .          .        2-2 

Tuberculous  meningitis  .          .          .          .         1-1 

Nephritis        .          .          .          .          .          .1-1 

Lead  poisoning       .          .          .          .          .0-3 

Amongst  200  cases  of  cerebral  tumour,  Paton  found  forty 
which  showed  no  signs  of  papilloedema  when  traced  either  to 
their  death  or  to  cure  by  operation,. 

He  showed  that  the  incidence  varies  greatly  with  the  site  of 
the  tumour. 

Thus  in  (tumours  limited  to  the  subcortical  region  or  to  the 
papilloedema  occurs  in  about  one-half  of  the  cases  only, 


and  when  it  occurs  it  seems  to  imply  that  the  subcortical 
tumour  has  invaded  the  basal  nuclei,  or  the  pontine  tumour  has 
invaded  the  cerebellum.  .S 

On  the  other  hand,  aircerebellar,  fourth  ventricle  and  tem- 
poro-sphenoidal  tumours  are  associated  with  papilloedema. 

Gowers,  speaking  of  tumours  arising  in  the  meninges,  states 
that  those  which  infiltrate  the  tissues,  and  those  which  develop 
at  the  base  of  the  brain,  are  more  apt  to  produce  papilloedema 
than  those  which  arise  on  the  convexity,  and  which  displace 
the  tissues  only. 

The  relative  immunity  from  papilloedema  in  tumours  of  the 
cortical  area  and  its  severity  in  tumours  of  the  cerebellum  was 
brought  out  by  Lawford  and.  Edmunds  in  their  analysis  of  107 
fatal  cases.2 

1  Bowman  Lecture,  Traw«.  Ophth.  /Soe.  U.K.,  1914,  xxxiv,  51. 

2  Trans.  Ophlh.  Koc.  U.K.,  1884,  iv.,  172,  and  v.,  184. 


INCIDENCE  19 

Papilloedema  is  of  exceedingly  rare  occurrence  in  cases  of 
cerebral  or  intra-cranial  haemorrhage  or  cerebral  softening. 

Amongst  sixty-eight  patients  whom  I  examined,  who  were 
diagnosed  by  the  physician  as  suffering  either  from  cerebral 
haemorrhage  or  softening,  I  did  not  find  a  case  of  papilloedema. 

Hughlings  Jackson1  says  :  "  Optic  neuritis  is  exceedingly  rare 
in  cases  of  extensive  destruction  of  brain  by  softening  or  clot. 
I  now  doubt  whether  I  have  ever  seen  double  optic  neuritis 
from  clot,  although  I  have  seen  the  two  things  coincident." 

Bristowe,2  however,  reports  a  case  in  which  the  opthalmo- 
scopic  appearances  were  confirmed  by  Nettleship,  and  in  which 
a  post-mortem  examination  was  made  by  Sharkey.  The 
haemorrhage  was  a  large  one,  involving  parts  of  the  optic 
thalamus,  internal  capsule,  lenticular  nucleus  and  the  white 
matter  of  the  temporo-sphenoidal  lobe.  The  brain  tissue  forming 
the  walls  of  the  cavity  was  examined  histologically  by  Sharkey, 
lest  it  should  be  a  tumour  into  which  haemorrhage  had  occurred, 
but  no  growth  was  found.  The  man  had  lived  for  forty-six 
days  after  the  onset  of  the  haemorrhage. 

In  cases  of  cerebral  syphilis  with  papilloedema,  in  65  per  cent, 
according  to  Uhthoff,  there  is  a  gummatous  syphilitic  tumour  of 
the  brain  or  its  meninges,  and  in  23  per  cent,  a  gummatous 
basal  meningitis. 

Recurrence. — It  is  rare  for  papilloedema  to  recur  after  once  it 
has  subsided,  even  though  atrophy  has  not  resulted. 

Taylor 3  reports  cases  of  recurrence  of  tumours  without 
recurrence  of  papilloedema. 

A  case  is  reported,  however,  by  de  Schwenitz  and  Thomson  4 
in  which  papilloedema  subsided  after  a  palliative  trephining, 
and  a  typical  post-neuritic  atrophy  resulted.  Thirteen  months 
later  papilloedema  recurred  with  5  D.  of  swelling. 

As  showing  for  how  long  a  time  a  cerebral  tumour  which 
ultimately  causes  papilloedema  may  be  present  before  this 
occurs,  Hughlings  Jackson  5  reports  the  case  of  a  man  who  had 

1  Trans.  Ophth.  Soc.  U.K.,  1881,  i.,  63. 

2  Trans.  Ophth.  Soc.  U.K.,  1886,  vi.,  363. 

3  Trans.  Ophth.  Soc.  U.K.,  1894,  xiv.,  111. 

4  Archiv.  of  Ophth.,  1895,  xxiv.,  252. 

5  Trans.  Ophth.  Soc.  U.K.,  1881,  i.,  80. 

2—a 


20  PAPILL(EDEMA 

had  symptoms  of  tumour  for  nine  years,  the  existence  of  which 
was  confirmed  post-mortem,  but  who  developed  papilloedema 
one  month  only  before  he  diedy 

Homolaterality. — Much  variation  of  opinion  has  been  ex- 
pressed by  different  observers,  as  to  whether  a  papilloedema  of 
earlier  development  or  greater  degree  on  one  side  implies  that, 
a  tumour  is  on  the  corresponding  side  of  the  brain. 

There  are  difficulties  in  this  matter.  In  the  first  place , 
papilloedema  commences,  as  a  rule,  so  nearly  contemporaneously 
in  each  eye,  that  what  small  difference  there  is  in  this  respect 
would  seldom  be  felt  to  have  much  weight. 

Again,  it  is  not  easy  in  most  cases,  when  the  papilloedema  is 
well  developed,  to  say  upon  which  side  it  is  the  more  advanced, 
and  measurements  of  the  degree  of  swelling  must  be  made  with 
meticulous  care  and  with  a  number  of  precautions,  if  they  are 
to  be  any  real  guide  in  the  matter. *• 

It  is  only,  I  believe,  by  making  a  diagram  of  the  disc  and 
vessels,  and  making  a  note  of  the  particular  spots  on  the  vessels 
which  are  used,  and  taking  these  same  spots  at  the  next  exami- 
nation, that  observations  of  any  value  can  be  arrived  at,  and 
it  is  seldom  that  a  difference  of  any  weight  can  be  estimated. 

I  have  known  observers  to  provide  measurements  of  the 
degree  of  swelling  of  the  disc  when  in  fact  they  were,  unknow- 
ingly, only  estimating  the  patient's  astigmatism.  Victor 
Horsley,  Gowers,  and  Gordon  Holmes  2  believed  that  the  tumour 
was  most  often  on  the  side  of  the  brain  on  which  the  earlier 
or  the  most  intense  papilloedema  was  present ;  Paton  and 
Farquhar  Buzzard,  on  the  other  hand,  believe  in  general  that 
no  such  relationship  exists.  Uhthoff  says :  "I  think  too  much 
importance  is  attached  by  some  surgeons  to  the  significance  of 
homolaterality  of  the  lesion  as  a  symptom." 

Mohr 3  found  that  in  56  per  cent,  of  cases  of  tumour,  papill- 
oedema was  ipso-lateral,  and  in  44  per  cent,  it  was  contra-lateral. 

There  is  no  doubt  that  in  very  many  cases  it  is  not  possible 
to  state  on  which  side  the  papilloedema  is  the  further  advanced  ; 

1  I  ',have  never  been  able  to  estimate  a  greater  degree  of  swelling  of  the  disc 
than  5-0  dioptres,  and  so  high  a  degree  as  this  is  very  exceptional. 

*  Trans.  Ophth.  Soc.  U.K.,  1908,  xxviii.,  134. 

*  Klin.  Monate.f.  Aug.,  1912,  xiv.,  401. 


DECOMPRESSION  OPERATIONS  21 

there  is  a  class  of  case,  however,  in  which  a  tumour  produces 
optic  atrophy  from  direct  pressure  on  the  optic  nerve  of  one 
side,  and  papillosdema  in  the  other  eye  from  the  increased  intra- 
cranial  pressure.  In  such  a  case  it  is  clear  that  the  tumour  is 
on  the  side  of  the  optic  atrophy. 

Cerebral  Abscess. — There  is  much  difference  of  opinion  as  to 
the  frequency  of  papillcedema  in  cases  of  cerebral  abscess,  and 
no  doubt  much  depends  upon  the  stage  at  which  it  is  seen. 

Gowers  l  believes  that  it  will  be  found  in  about  75  per  cent, 
of  cases  where  there  is  reason  to  believe  that  the  abscess  has 
existed  for  four  weeks  or  longer. 

There  seems  to  be  a  consensus  of  view,  that  the  existence  of 
unilateral  disc  changes,  or  of  a  greater  degree  of  change  on  one 
side  than  on  the  other,  does  in  the  case  of  abscess,  imply  with 
much  likelihood  that  the  abscess  is  on  that  same  side  of  the 
brain. 

Thus  Uhthoff  2  says  that  in  13  per  cent,  of  cases  of  cerebral 
abscess  with  papillcedema,  the  papillcedema  was  unilateral, 
and  that  in  four-fifths  of  these  it  was  on  the  same  side  as  the 
abscess,  and  further,  that  where  it  was  bilateral,  the  greater 
prominence  was  on  the  side  of  the  abscess  in  four-fifths  of  the 
cases,  a  view  which  is  similar  to  that  held  by  Gowers. 

The  Value  of  Cerebral  Decompression  Operations  for  the 
Preservation  of  Sight. — It  is  well  recognised  that  a  high  degree 
of  papillcedema  may  be  present  without  the  visual  acuity  or 
the  visual  fields'^being  impaired,  and  that  the  process  may  run 
its  course  to  complete  subsidence  and  leave  the  visual  functions 
intact. 

On  the  other  hand^most  cases  of  papillcedema  are  followed 
by  a  varying  degree  of  atrophy  of  the  nerve,  and  greater  or  less 
impairment  of  sight,  so  that  if  it  can  be  shown  that  a  palliative 
decompression  operation  will  lessen  the  likelihood  of  ensuing 
blindness,  even  though  no  other  advantage  were  obtained,  its 
performance  is  clearly  justified. 

Of  the  benefit  to  be  obtained  in  this  respect,  however,  there 
can  be  no  doubt. 

1  "  Medical  Ophthalmoscopy,"  1904,  147. 

2  Bowman  Lecture,  Trans.  Ophth,  Soc.  U.K.,  1914,  xxxiv.,  65. 


22  PAPILL(EDEMA 

The  general  view  is  that  the  earlier  the  operation  is  per- 
formed the  better  is  the  prospect,  and  that  in  the  presence  of 
a  cerebral  tumour,  trephining  should  be  done  on  the  first 
symptom  of  visual  defect,  such  as  attacks  of  temporary  blind- 
ness, even  though  papillcedema  is  absent.  The  ultimate  con- 
dition of  the  sight  in  unoperated  cases  is  analysed  by  Uhthoif.1 
He  finds  that  in  15  percent,  of  cases  the  sight  remained  nearly 
normal  for  periods  up  to  six  years,  and  in  another  28  per  cent, 
diminished  sight  remained  stationary  for  periods  up  to  five  years  ; 
in  38  per  cent,  there  was  great  loss  of  sight  up  to  blindness  ; 
19  per  cent,  were  untraced.  He  points  out  that  in  those  cases 
in  which  the  papillcedema  remains  apparently  unchanged  for 
long  periods,  e.g.,  a  year  or  more,  if  deterioration  of  sight  then 
sets  in,  its  progress  is  usually  rapid  and  complete. 

In  those  cases  in  which  operation  was  performed  late,  when 
atrophic  changes  were  clearly  present,  50  per  cent,  went  blind 
in  spite  of  trephining. 

In  cases  in  which  operation  is  done  early,  de  Schweinitz  and 
Holloway  2  show  how  good  are  the  results,  for  in  an  analysis 
of  212  operations  they  find  that  vision  was  preserved  in  76-5 
per  cent. 

v.  Hippel,3  as  a  result  of  the  study  of  272  cases,  says  it  can 
be  concluded  with  certainty,  that  choked  disc  will  disappear 
after  operation  in  the  course  of  some  weeks,  and  that  as  a  rule 
vision  continues  to  be  good  if  it  was  satisfactory  before,  but 
that  it  does  not  recover  if  it  was  greatly  deteriorated. 

Contraction  of  the  field  is  an  unfavourable^sign.  He  urges 
that  operation  should  be  done  early,  for  cases  in  which  retention 
of  vision  occurs  without  operation  are  not  of  sufficient  frequency 
to  justify  delay. 

He  advises  that  the  operation  be  done  in  two  stages,  the  first 
consisting  of  removal  of  bone,  and  the  second  of  incision  of  the 
dura. 

De  Schweinitz  advises  against  trephining  for  syphilitic 
lesions  unless  a  six  or  eight  weeks'  course  of  medicinal  treat- 

1  Trans.  Ophth.  Soc.  U.K.,  Bowman  Lecture,  1914,  xxxiv.,  84. 

2  Annals  of  Ophth.,  1911,  xx.,  272. 

"  Cerebral  Decompression  in  Choked  Disc,"  Leipsic,  1909. 


LUMBAR  PUNCTURE  23 

ment  has  failed  ;    he  also  considers  it  inadvisable  to  trephine 
for  the  papill oedema  of  renal  disease. 

It  seems  abundantly  clear,  that  a  decompression  operation     t^/^ 
is  advisable  in  cases  of  cerebral  tumour  associated  with  papill-   ' 
oedema  if  only  for  the  preservation  of  sight,  and  that  the  earlier 
the  operation  is  performed,  the  better  is  the  prospect  of  a  good 
and  permanent  result. 

Lumbar  Puncture. — There  is  some  evidence  to  show  that 
lumbar  puncture  may  result  in  a  reduction  of  the  swelling  of 
the  disc  in  cases  of  cerebral  tumour,  though  this  procedure  is 
not  without  definite  immediate  risks  to  life,  and  fresh' retinal 
haemorrhages  may  be  produced  by  it.1 

Uniocular  Papillcedema. — In  a  few  cases  papillcedema  runs 
its  course  to  complete  subsidence  in  one  eye,  the  other  remaining 
normal  throughout,  and  it  seems  difficult  in  such  a  case  to  avoid 
the  conclusion  that  it  is  dependent  upon  disease  either  in  the 
optic  nerve  itself  or  in  the  orbit  in  proximity  to  the  nerve. 

NYSTAGMUS. 

Nystagmus  occurs  under  many  widely  differing  conditions 
and  obtrudes  itself  to  the  notice  of  workers  in  several  branches 
of  medicine  and  surgery.  It  is  familiar  to  the  physician,  the 
neurologist,  the  aurist  and  the  ophthalmic  surgeon. 

Certain  more  or  less  rhythmic  movements  of  the  eyes  occur 
which  closely  simulate  nystagmus  and  may  be  termed  "  nystag- 
moid  ;  "  they  have  not  the  significance  of  true  nystagmus  and 
may  at  once  be  disposed  of.  They  are  sometimes  referred  to  as 
pseudo -nystagmus . 

Pseudo-Nystagmus  is  seen  when,  the  eyes  having  been  carried 
almost  to  the  extreme  limit  of  their  movement  in  some  direction, 
a  sustained  straining  effort  to  increase  the  movement  in  the 
same  direction  is  made.  The  result  is  a  succession  of  jerky 
movements  continuing  the  direction  of  the  original  movement, 
and  returning  again  to,  but  not  beyond,  the  point  from  which 
the  additional  movement  was  started. 

Selbourne  Bailey  2  showed  that  of  500  normal  individuals 

1  Spiller  and  de  Schweinitz,  Jour,  of  Nervous  and  Mental  Dis.,  1917,  xlvi.,  10. 

2  Quoted  by  Scott,  Trans,  Boy.  Soc.  Med.,  Sect,  of  Neurol.  Ophth.  Otol.,  1913, 
vii.,  xl- 


24  NYSTAGMUS 

20  per  cent,  showed  nystagmus  of  this  type  when  the  visual 
axes  deviated  beyond  the  range  of  binocular  vision. 

True  Nystagmus. 

The  movements  may  be  horizontal,  vertical,  rotatory  or 
mixed,  but  in  rare  instances  other  movements  than  these  occur. 

Thompson  and  Spicer  state  that  in  infants  with  spasmus 
nutans,  the  eyes  occasionally  undergo  a  rhythmic  alternate 
divergence  and  convergence  ;  Maddox  l  exhibited  a  case  of 
see-saw  nystagmus,  one  eye  moving  vertically  upwards  whilst 
the  other  moved  downwards ;  the  patient  suffered  from 
bitemporal  hemianopia. 

From  his  investigations  of  the  eye  movements  in  the  rhyth- 
mic nystagmus  which  follows  rapid  spinning  in  a  chair,  Adolphe 
Abrahams  2  showed  by  means  of  cinematography,  that  the 
movements  were  at  the  rate  of  four  a  second,  and  that  the  rate 
of  the  rapid  movement  is  to  the  return  movement  as  three  is  to 
five. 

Snell  estimated  the  movements  in  miner's  nystagmus  as 
from  60  to  100  per  minute,  and  Dransart  at  from  80  to  140. 

Wilfred  Harris  3  reports  a  unilateral  case  in  which  the  move- 
ments averaged  300  a  minute,  and  in  cases  of  voluntary  nystag- 
mus the  movements  are  so  rapid  that  their  rapidity  can  hardly 
be  estimated. 

Of  the  common  varying  movements  which  occur,  the  hori- 
zontal is  by  a  long  way  the  most  common.  Spicer  4  analysed 
200  cases  and  found  the  following  proportions  :— 

Per  cent. 

Horizontal  .          .-         .          .          .50 

Rotatory    ......      15 

Vertical      ......      12 

Mixed         ......       4 

Irregular    .          .          .          .  .2 

Circumductory    .....       2 

Disjunctive          .....        1 

1  Pro.  Roy.  Soc.  Med.,  Sect,  of  Neurol.  Ophth.  Otol.,  1913,  vii.   xii. 

2  Pro.  Roy.  Soc.  Med.,  Sect,  of  Neurol.  Ophth.  Otol.,  1913,  vii.,  Ixvii. 

3  Pro.  Roy.  Soc.  Med.,  Sect,  of  Neurol.  Ophth.  Otol ,  1913,  vii.,  i. 

*  Pro.  Roy.  Soc.  .Med.,  Sect,  of  Neurol.  Ophth.  Otol.,  1913,  vii.,  xxi 


UNILATERAL  25 

Unilaterality. — In  a  few  cases  the  movements  may  be  limited 
to  one  eye,  or  they  may  be  present  only  when  one  eye  is  covered. 
In  some  cases  of  apparent  unilateral  nystagmus  it  will  be 
found  on  minute  examination  that  the  other  eye  is  not  really 
quite  steady,  but  that  very  fine  movements  are  present  which 
are  detected  by  ophthalmoscopic  examination. 

Duane  1  collected  52  published  cases  of  unilateral  nystagmus, 
and  of  these 

34  were  vertical, 
11  were  horizontal, 
5  were  rotatory, 
2  were  mixed. 

It  will  be  noticed  that  here  the  vertical  movements  are  more 
common  than  the  horizontal.  As  features  of  these  cases  he 
mentions  : — 

(1)  Apparent  oscillation  of  objects  looked  at. 

(2)  Oscillatory  diplopia. 

(3)  The  patient  can  himself  see  the  movements  of  the  eye  in 

a  mirror. 

Heimann  2  divides  unilateral  cases  into  those — 

(1)  Occurring  in  highly  amblyopic  and  misdirected  eyes. 

(2)  Occurring  in  the  course  of  severe  nervous  disease. 

(3)  Of    a    transitory    nature    accompanying    or    following 

spasmus  nutans. 

Neustatter  3  showed  that  the  movements  will  often  occur  in 
the  other  eye  if  the  first  one  is  covered. 

Symptoms  of  Nystagmus. — In  spite  of  the  rapid  eye  move- 
ments, objects  do  not  in  most  cases  appear  to  the  patient  to 
be  moving,  but  in  miner's  nystagmus,  and  nystagmus  of 
labyrinthine  origin,  such  apparent  movement  does  occur  and 
gives  rise  to  great  confusion. 

Most  patients  cannot  see  the  movement  of  their  eyes  in  a 
mirror.  Some  say  they  can  at  times  feel  the  movements. 

It  has  been  pointed  out  by  Soelberg-Wells  and  Duane  that 

1  Trans.  Amer.  Ophth.  Soc.,  1906.,  xi.,  63. 

2  Klin.  Monate.  f.  Augenheilk.,  1902,   ii.,  99. 

3  Archives  of  Ophth.,  1907,  xxx.,  158. 


26  NYSTAGMUS 

some  patients  with  horizontal  nystagmus  read  more  comfortably 
and  with  less  confusion  if  the  book  be  held  on  one  side,  so  that 
the  lines  of  print  run  vertically.  I  have  seen  a  medical  man 
whose  only  eye  was  highly  astigmatic  make  use  of  the  same 
manoeuvre. 

Vertigo  of  ocular  origin  is  marked  in  miner's  nystagmus. 

Visual  Acuity. — Nystagmus  itself  does  not  necessarily  pro- 
duce poor  sight,  the  acuity  may  be  normal.  I  have  known  a 
man  carry  on  the  work  of  a  banker's  clerk,  entailing  the  constant 
addition  of  columns  of  figures,  without  serious  trouble. 

In  many,  however,  the  sight  is  very  defective,  either  from  the 
lesion  which  has  produced  the  nystagmus,  or  from  associated 
defects  such  as  albinism  or  high  refractive  errors. 

Variations. — Apart  from  the  variations  produced  according 
to  the  position  of  the  -eyes,  by  covering  one  eye,  by  fixing  the 
head  in  spasmus  nutans,  or  other  obvious  reasons,  spontaneous 
alterations  of  amplitude  of  the  movements  occur  for  which  no 
cause  can  be  found. 

Fawcett  and  Ormond  report  a  case  1  in  which  the  movements 
were  sometimes  vertical  and  at  others  horizontal. 

The  Causes  of  Nystagmus. — These  may  be  conveniently 
classed  under  the  following  heads  :— 

(A)  Hereditary  nystagmus. 

(B)  Voluntary  nystagmus. 

(C)  Nystagmus  resulting  from  marked  bilateral  visual  defect 

originating  very  early  in  life. 

(D)  Occupational  nystagmus. 

(E)  Nystagmus    as    a    manifestation    of     general    nervous 

disease. 

(F)  Labyrinthine  nystagmus. 

(A)  Hereditary  nystagmus. — This  heading  will  comprise  those 
cases  which  date  from  birth  and  have  an  hereditary  tendency  : 
several  members  of  the  family  may  be  affected. 

Here  would  be  included  cases  of  albinism  which  cannot  be 
missed,  but,  in  addition,  there  would  be  included  those  cases 
of  partial  albinism  which  in  the  individual  may  easily  be 

1  Pro.  Roy.  Soc.  Med.,  Sect,  of  Neurol.  Ophth.  Otol.,  1913,  vii.,  xvi. 


HEREDITARY  27 

overlooked,  the  cause  of  which,  however,  becomes  plain  on  the 
discovery  of  clear  cases  of  albinism  in  the  same  family. 

The  supposition  that  albinism  may  involve  a  part  of  the  eye, 
e.g.,  the  retinal  epithelium  at  the  macula — and  may  so  be 
responsible  for  nystagmus,  although  all  other  certain  signs  of 
albinism  in  the  individual  are  missing — was  first  advanced  by 
Nettleship.1 

He  says  2  :  "  The  hypothesis  is,  that  the  imperfect  sight,  and 
with  it  the  nystagmus,  is  caused  by  deficiency  of  pigment  in  the 
retinal  epithelium  ;  that  this  want  may  vary  in  degree,  and  may 
even,  perhaps,  affect  only  a  part — say  the  central  region — of  the 
fundus  ;  and,  lastly,  that  such  relative  or  absolute  lack  of 
pigment  in  the  epithelium  is  not  recognisable  with  any  certainty 
by  ophthalmoscopic  examination,  the  different  depths  of  tint 
at  the  fundus  depending  far  more  upon  differences  of  pigmenta- 
tion of  the  choroid,  than  of  the  hexagonal  epithelium." 

Nettleship  points  out  that  in  some  pedigrees  of  hereditary 
nystagmus  both  sexes  are  affected,  descent  being  through  the 
unaffected  mother ;  further,  that  in  some  pedigrees,  oscillatory 
head  movements  are  associated  with  the  nystagmus,  whilst  in 
others  there  is  no  such  association. 

(B)  Voluntary  nystagmus. — A  few  individuals  can  produce 
nystagmus  at  will. 

I  have  seen  a  medical  man  who  could  do  this  in  a  very 
striking  manner — he  discovered  it  by  chance  when  a  boy ;  the 
movements  were  almost  inconceivably  rapid,  a  fact  which  has 
been  noted  in  other  reported  cases,  and  the  term  "  shuddering 
of  the  eyes  "  describes  rather  well  the  effect  produced. 

Von  Graefe  believed  that  it  was  only  those  who  had  at  one 
time  suffered  from  involuntary  nystagmus  that  could  reproduce 
it  in  this  way,  but  the  instance  referred  to  above  refutes  the 
contention. 

Williams  3  reports  the  case  of  a  man  of  forty-five  who  could 
give  his  eyes  "  an  almost  inconceivably  rapid  oscillatory 
movement  in  any  direction  at  pleasure." 

1  Roy.  London  OpJith.  Hospital  Reports,  1902,  xv.,  110. 

2  Bowman  Lecture,  Trans.  Ophth.  Soc.  U.K.,  1909,  xxix.,  125. 

3  Roy.  London  Oph.  Hosp.  Reports,  1866,  v.,  223. 


28  NYSTAGMUS 

(C)  Marked  amblyopia  due,  to  bilateral  ocular  defects  in  very 
early  life. — The  most  common  causes  of  such  amblyopia  are 
dense  corneal  opacities,  especially  those  caused  by  ophthalmia 
neonatorum,  and  congenital  cataract. 

Of  1,000  children  with  nystagmus  due  to  corneal  nebulae, 
Bishop  Harman  l  found  that  the  nebulae  in  85  per  cent,  were 
due  to  ophthalmia  neonatorum,  and  of  ninety  children  blinded 
by  this  disease,  every  one  showed  nystagmus. 

He  also  states  that  50  per  cent,  of  cases  of  posterior  uveitis 
develop  nystagmus,  and  that  nystagmus  only  appears  in 
interstitial  keratitis  when  posterior  uveitis  also  exists. 

It  is  probable  that  there  is  a  limited  period  during  the  first 
few  months  of  life,  during  which  complete  co-ordination  of  eye 
,  movements  can  be  acquired,  and  if,  during  this  time,  such 
co-ordination  is  not  developed,  owing  to  enforced  closure  of  the 
eyes  from  ophthalmia  neonatorum  or  some  similar  disease,  then 
it  is  never  properly  developed  at  all. 

Thus  one  may  see  children  with  nystagmus  as  a  legacy  from 
an  attack  of  ophthalmia  neonatorum,  in  whom  the  ocular  media 
are  all  but  perfectly  clear. 

The  mode  of  development  of  this  type  of  nystagmus  is  thus 
given  by  Spicer  2  : — 

"  When  a  child  is  born  the  eyes  move  independently  and 
wander  about  till  something,  such  as  a  light,  attracts  the  vision, 
the  image  approaches  the  fovea  and  becomes  more  defined,  and 
when  it  reaches  the  fovea  becomes  sharply  defined  and  clear  to 
the  normal  eye ;  the  muscles  and  visual  centres  quickly 
co-ordinate  themselves,  so  that  without  any  conscious  effort  the 
child  can  bring  the  image  of  the  object  on  the  fovea  at  once. 

"  Further,  all  the  movements  of  the  eyes  become  co-ordinated, 
although,  to  do  this,  many  muscles  whose  actions  are  not  the 
same  in  the  two  eyes,  are  brought  into  simultaneous  use. 

'  The  proper  action  of  the  co-ordinating  centre  of  muscular 
action  requires  the  stimulus  of  exact  retinal  images  ;  without 
proper  retinal  stimulation  fixation  is  imperfect. 

"  Among  the  actual  causes  which  produce  imperfect  images, 

1  Pro.  Roy.  Soc.  Med.,  Sect.  Neurol.  Ophth.  Otol.,  1913,  vii.,  Ivi. 

2  Pro.  Roy.  Soc.  Med.,  Sect.  Neurol.  Ophth.  Otol.,  1913,  vii.,  xxiv. 


OCCUPATIONAL  29 

the  most  frequent  are  the  consequences  of  ophthalmia  neona- 
torum,  anterior  synechia,  pyramidal  cataract,  and  congenital 
cataract. 

"  The  failure  of  use  of  the  eyes  in  the  early  days  of  life  itself 
leads  to  imperfection  in  the  development  of  the  visual  centre, 
and,  consequently,  of  the  co-ordinating  centres  of  the  muscles 
moving  the  eyes. 

"  These  children  remain  amblyopic  and  have  oscillating 
eyes. 

"  That  this  want  of  development  of  the  visual  and  co- 
ordinating centres  is  due  to  a  want  of  use  during  the  early 
stages  of  life,  is  shown  by  the  fact  that  leukomata  and  cataract 
acquired  in  after-life  do  not  lead  to  amblyopia  or  nystagmus." 

(D)  Occupational  nystagmus. — This  term  comes  to  be  almost 
synonymous  with  miner's  nystagmus,  for,  although  nystagmus 
may  develop  in  those  engaged  in  a  few  other  occupations,  as 
pointed  out  by  Snell,  it  is  exceedingly  infrequent  and  of  less 
importance. 

The  special  features  of  this  form  of  nystagmus  are  the  very 
marked  subjective  symptoms  which  accompany  it ;  thus 
Llewellyn  l  remarks  that  nystagmus  in  general  is  conspicuous 
as  a  physical  sign  rather  than  for  its  subjective  symptoms, 
whereas  in  miner's  nystagmus  it  is  the  symptoms  that  are 
distressing  and  disabling. 

Figures  as  to  incidence  are  of  little  value,  for  there  are 
characters,  local  to  particular  types  of  mines,  which  are  very 
largely  responsible  for  its  occurrence  or  its  absence. 

Llewellyn  states  that  during  1912  one  man  in  every  2,700 
employed  underground  received  compensation. 

Causation. — Certain  facts  as  to  its  causation  are  clear,  but 
with  regard  to  others  there  remains  considerable  divergence  of 
views  amongst  those  who  have  made  a  particular  study  of  the 
condition,  even  where  what  would  seem  discrete  facts  are 
concerned. 

There  seems  to  be  a  complete  consensus  of  view,  that  in 
mines  where  it  is  possible  to  use  naked  lights  instead  of  the  Davy 
lamps  the  condition  is  almost  unknown,  and  Llewellvn  has 

1  Pro.  Roy.  Soc.  Med.,  Sect.  Neurol.  Ophth.  Otol.,  1913,  vii.,  xliii. 


30  NYSTAGMUS 

pointed  out  how  exceedingly  feeble  is  the  illumination  obtained 
from  a  Davy  lamp,  and  how  little  of  its  light  reaches  the  eye, 
having  regard  to  the  dead  black  surroundings  which  absorb 
most  of  the  light. 

Now  it  is  the  higher  visual  acuity  obtained  at  the  f  ovea  which 
is  mainly  responsible  for  fixation  ;  with  dark  adaptation, 
however,  the  acuity  at  the  fovea  becomes  less  than  that  of  the 
rest  of  the  retina,  which  is  uniform  all  over. 

It  is  easy  to  satisfy  oneself  with  regard  to  this  point  by 
looking  at  a  star  of  low  intensity  on  a  dark  night. 

It  is  seen  much  more  distinctly  if  the  line  of  vision  is  directed 
to  one  side  of  it,  so  as  to  bring  its  image  on  some  part  of  the 
retina  away  from  the  fovea  instead  of  directly  on  this  spot. 

Owing,  then,  to  the  absence  of  a  spot  of  hyperacute  vision, 
fixation  becomes  difficult,  the  eyes  are  loosed  from  the  control 
of  central  vision,  and  wandering  movements  easily  arise. 

Nieden  l  and  Snell  agree  in  looking  upon  the  forced  use  and 
over-fatigue  of  the  elevator  muscles  of  the  eyes  as  one  of  the 
most  important  causes. 

They  state  that  it  chiefly  occurs  in  men  engaged  in  "  holing," 
i.e.,  in  obtaining  coal  by  undercutting  masses  ;  this  entails  a 
lying  position,  the  eyes  being  directed  upward  and  obliquely 
all  the  time  ;  the  miner  is  continuously  moving  his  eyes  right 
and  left,  but  always  upwards,  till  the  hole  is  completed,  and 
this  puts  a  very  great  strain  on  the  elevator  muscles  ;  a  view 
which  is  also  shared  by  Lafon.2 

Reid,3  however,  shows  that  it  may  occur  in  pits  where  men 
do  not  lie  down,  and  where  no  holing  is  done. 

He  looks  upon  the  following  as  important  factors  :— 

(1)  Conditions  tending  to  do  away  with  yellow  spot  fixation, 

i.e.,  feeble  illumination. 

(2)  Conditions  tending  to   disturb   the   equilibrium   of   the 

body. 

(3)  Conditions  in  which  a  series  of  rhythmic  movements  is 

"  Miner's  Nystagmus,"  Bergmann,  Wiesbaden. 
-  Annnlf*  tTOeniKstique,  1920,  clvii.,  529. 
3  lirain,  1906,  xxix,  367. 


OCCUPATIONAL  31 

made  by  the  body,  the  eyes  remaining  fixed  on  the 
object. 

(4)  Debilitating  influences,  alcoholism,  influenza,  accidents, 
etc. 

Llewellyn  considers  the  presence  of  refractive  errors  an 
important  contributory  cause,  whilst  Nieden  thinks  refractive 
errors  have  but  little  influence. 

Llewellyn  states  that  of  887  men  with  nystagmus  examined 
by  him,  a  much  larger  proportion  than  of  normal  miners  had  fair 
hair. 

The  Movements. — The  movements  are  rotatory  or  lateral  in 
most  cases  ;  they  are  increased  on  looking  upwards,  and 
decreased  or  even  abolished  by  looking  down,  and  as  a  conse- 
quence of  this  the  men  will  often  walk  about  with  the  head 
thrown  back,  their  eyes  being  thus  brought  into  the  most  stable 
position.  ^_^ 

Diplopia  is  a  not  uncommon  symptom,  and  in  most  cases  is 
crossed,  and  so  due  to  defects  of  convergence  ;  thus,  of  seventy- 
one  cases,  Dransart  l  found  crossed  diplopia  in  sixty-nine  and 
homonymous  diplopia  in  two  ;  and  Ohm,2  amongst  195  cases, 
found  crossed  diplopia  in  188  cases  and  homonymous  in  seven 
only. 

(E)  There  are  several  diseases  of  the  nervous  system  in 
which  nystagmus  frequently  occurs,  such  are  disseminate 
sclerosis,  syringomyelia,  and  Friedreich's  ataxia  ;  these  are 
referred  to  under  their  individual  heads. 

Lesions  of  the  cerebellum,  whatever  their  nature,  whether 
inflammatory  or  neoplasms,  are  very  frequently  associated  with 
nystagmus. 

Uhthoff  3  found  that  nystagmus  occurred  in  17  per  cent,  of 
intra -cranial  tumours,  and  that  75  per  cent,  of  these  were 
situated  in  the  posterior  cranial  fossa  ;  and  Taylor  states  that 
lesions  of  the  cerebral  hemispheres  do  not  give  rise  to  nystagmus. 
Risien  Russell  (quoted  by  Taylor)  has  shown  that,  after 
removal  of  one-half  of  the  cerebellum  in  animals,  the  eyes  move 

1  Annales  (TOculistique,  1877,  Ixxviii.,  116. 

2  "  Miner's  Nystagmus,"  Leipzig,  1912. 

3  Bowman  Lecture,  Trans.  Ophth.  Soc.  U.K.,  1914,  xxxiv.,  Ixxii. 


32  NYSTAGMUS 

in  coarse,  slow  jerks  when  they  are  directed  towards  the  side 
of  the  lesion,  and  in  more  rapid  jerks  of  smaller  range  when 
they  are  turned  away  from  the  lesion. 

This  observation  has  been  confirmed  clinically  in  man,  in  cases 
of  cerebellar  disease,  by  Russell  and  others  ;  and  in  cerebellar 
and  extra-cerebellar  abscess  by  Sydney  Scott.1 

According  to  James  Taylor,  nystagmus  may  occur  as  a 
result  of  peripheral  lesions  in  the  nerves,  as  in  alcoholic  neuritis, 
or  in  the  muscles,  as  in  myasthenia  gravis. 

Lenoble  and  Aubineau,2  under  the  term  "  Myoclonic 
Nystagmus,"  report  a  group  of  cases  which  is  characterised  by 
nystagmus  associated  with  spasmodic  movement  of  the  head 
and  body,  quivering  of  the  muscles,  and  increased  knee-jerks. 

It  affects  several  generations,  and  may  be  accompanied  by 
mental  and  physical  deterioration. 

(F)  Labyrinthine  Nystagmus. — In  disease  of  the  internal 
ear  in  which  the  semi-circular  canals  are  involved  nystagmus 
becomes  a  prominent  feature. 

It  can  also  be  elicited  in  normal  individuals  by  excessive 
stimulation  of  the  labyrinth,  as  by — 

(1)  Syringing  the  external  ear  with  hot  or  cold  water. 

(2)  By  rotating  the  patient  in  a  chair  especially  designed  for 

this  purpose. 

(3)  By  passing  a  galvanic  current  through  the  head. 

The  resulting  nystagmus  is  rhythmic,  having  a  rapid  and  a 
slow  component,  is  bilateral,  and  is  horizontal  or  rotatory  in 
type.3 

If  nystagmus  be  elicited  by  means  of  rotation  in  the  rotatory 
chair,  one  or  other  of  the  semi-circular  canals  can  be  stimulated 
at  will  according  to  the  position  in  which  the  head  is  placed, 
and  the  corresponding  nystagmus  produced. 

The  nystagmus  is  seen  on  stopping  the  rotation,  and  in 
direction  is  the  opposite  of  that  of  the  previous  rotation  (Scott). 

Fridenberg  4  says  :    "It  has  been  shown  that  stimulation  of 

1  Pro.  Roy.  Soc.  Med.,  Sect.  Neural.  Ophth.  Otol.    1913,  vii,  xli. 
*  Rev.  de  Med.,  1911,  xxxi.,  209. 

8  Scott,  Pro.  Roy.  Soc.  Med..  Sect.  Neurol.  Ophth.  Otol.   1913.  vii.  xl. 
«  Trans.  Amer.  Ophth.  flor..  1891,  xi,  571. 


LABYRINTHINE  33 

the  nerve  terminals  in  the  ampulla  of  a  semi-circular  canal 
produces  a  sensation  of  turning  in  the  plane  of  that  canal, 
and  that  the  resultant  nystagmus  corresponds  to  the  change  of 
position  of  objects  which  would  follow  such  a  rotation  of  the 
individual." 

'  The  sensation  of  turning  is  always  towards  the  irritated 
side,  and  the  rapid  movement  of  the  nystagmus  is  towards  this 
side. 

"  In  labyrinthine  nystagmus,  indicating  irritation,  nystagmus 
is  increased  on  looking  towards  the  affected  side,  and  may 
disappear  entirely  on  looking  in  the  opposite  direction." 

Nystagmus  can  be  evoked  momentarily  in  some  patients 
with  middle -ear  disease  associated  with  a  fistulous  communica- 
tion between  the  middle  and  internal  ears. 

This  is  done  by  sudden  compression  of  the  meatal  contents. 

It  is  essential  that  the  neuroepithelium  of  the  vestibule 
should  be  intact,  and  that  the  local  condition  is  favourable  to 
the  transmission  of  sudden  changes  of  pressure  to  the  interior 
of  the  labyrinth  ;  this  is  known  as  the  "  fistel-symptom." 

Immediately  one  labyrinth  is  rendered  functionless,  spon- 
taneous rhythmic  nystagmus  towards  the  opposite  side  occurs  ; 
this,  however,  at  once  ceases  if  the  other  labyrinth  is  destroyed 
(Scott). 

Head  Nodding  and  Nystagmus  (Spasmus  Nutans). — In  small 
infants  under  a  year  old  an  association  of  nystagmus  with 
rhythmic  nodding  or  shaking  movements  of  the  head  is  some- 
times seen.  It  is  believed  by  some  to  be  due  to  the  children 
having  been  kept  in  ill-lit  rooms.  It  occurs  in  the  first  year 
of  life,  and  is  more  frequent  in  females. 

The  head  movements  precede  the  nystagmus  by  some  weeks, 
whilst  the  nystagmus  persists  for  a  number  of  weeks  after  the 
head  movements  have  ceased.  The  movements  cease  during 
sleep.1  There  is  no  associated  disease  of  the  eyes  or  nervous 
system,  and  the  condition  is  always  recovered  from. 

Distinct  from  the  foregoing,  Hancock  2  has  reported  a 
congenital  and  hereditary  form  of  head  nodding  with  nystagmus 

1  Madden,  Lancet,  1890,  i,  1294. 

2  Roy.  Lond.  Ophth.  Hosp.  Reports,  1907-8,  xvii.,  112. 


34 


RETINAL  HEMORRHAGES 


which  commences  at  birth,  persists  throughout  life,  and  is 
associated  with  defective  sight.  In  one  family  he  found  six 
out  of  nine  children  affected. 

RETINAL   HAEMORRHAGES   IN   GENERAL. 

Many  and  varied  are  the  causes  of  retinal  haemorrhages,  and 
whilst  in  some  the  immediate  cause  is  readily  appreciated,  this 
is  not  so  in  all  instances. 


FIQ.  11. — Showing  haemorrhages  into  the  nerve  fibre  layer  of  the  retina 
which  mark  out  very  clearly  the  texture  of  this  layer.  From  a 
woman  of  fifty  years  of  age  whose  systolic  blood 'pressure  w;i.- 
285  mm. 

It  is  only  proposed  to  offer  a  few  general  remarks  here  upon 
retinal  haemorrhages  as  such. 

They  vary  greatly  in  number,  in  size,  as  to  their  position  in 
the  retina,  whether  near  the  disc  or  peripherally  placed,  and  as 
to  the  layers  of  the  retina  involved. 

They  are  usually  most  extensive  over  the  central  regions 
where  the  retina  is  thickest  and  the  largest  vessels  are 
placed,  and  to  the  temporal  rather  than  the  nasal  side  of 
the  disc. 

As  to  site,  it  is  to  be  remembered  that  the  retinal  vessels  do 


SUBHYALOID 


35 


not  penetrate  deeper  than  the  external  molecular  layer,  and 
consequently  haemorrhages  will  tend  to  be  most  frequent  in  the 
inner  layers  of  the  retina,  but  larger  effusions  may  infiltrate  all 
the  layers  ;  the  most  external  retinal  layers  are  nourished  by 
the  choroid  and  in  some  animals  no  retinal  vessels  exist. 

A  special,  type  of  haemorrhage  is  that  which  occurs  in  the 
yellow  spot  region  between  the  nerve  fibre  layer  and  the 
internal  limiting  membrane,  it  is  of  the  shape  of  a  half-moon 


FIG!  12. — A  gross  haemorrhage  of  heterogeneous  texture,  which  was  pro- 
bably infiltrating  all  the  layers  of  the  retina,  in  a  woman  whose  systolic 
blood  pressure  was  205  mm.  The  second  drawing  (A')  was  made 
two  years  and  six  weeks  after  the  first ;  the  retina  over  the  area 
previously  involved  showed  a  few  minute  bright  dots,  but  otherwise 
no  sort  of  abnormality  was  present ;  there  was  no  residue  of  the 
previous  haemorrhage.  There  was  nothing  to  indicate  with  certainty 
the  source  of  the  previous  bleeding,  but  the  artery  which  traverses 
the  area  showed  marked  variability  of  calibre. 

with  a  perfectly  sharp  horizontal  upper  margin.  It  is  spoken 
of  as  a  subhyaloid  haemorrhage  ;  I  have  seen  this  type  of 
haemorrhage  on  three  occasions  in  leukaemia. 

When  effusion  of  blood  takes  place  in  the  nerve  fibre  layer 
it  shows  up  the  texture  of  this  layer  ;  the  haemorrhages  resulting 
are  often  likened  to  tongues  of  flame,  and  so  are  spoken  of  as 
"flame-shaped"  (Fig  11). 

When  the  haemorrhages  are  in  the  deeper  parts  of  the  retina 

3—2 


36 


RETINAL  HEMORRHAGES 


they  tend  to  be  roughly  circular  in  outline,  and  do  not  show 
the  striate  texture  of  the  more  superficial  flame-shaped  ones. 


-z D 


FIG.  13. — A  composite  diagram  to  indicate  the  length  of  time  required  for 
the  disappearance  of  different  types  of  retinal  haemorrhage.  The 
haemorrhages  of  different  types  have  been  collected  from  their  proper 
notes  and  incorporated  here.  The  time  given  dates  from  the  occasion 
when  the  haemorrhage  was  first  seen.  A.  A  small  deep  haemorrhage  ; 
it  had  gone  in  eight  weeks.  B.  A  flame-shaped  haemorrhage  ;  no 
trace  was  left  in  thirty  days.  C.  An  irregular  hoemorrhage  which 
disappeared  in  seven  weeks.  D.  What  appeared  to  be  small  globules 
of  blood  in  the  retina  ;  they  were  still  present,  and  but  little,  if  at  all, 
changed  two  years  and  ten  months  later.  (I  have  several  times 
seen  blood  in  relation  with  the  non-vascular  parts  of  the  eye,  the 
vitreous  or  lens  capsule,  remain  bright  red  and  apparently  unchanged 
for  long  periods  up  to  six  years.)  E.  The  area  was  mottled  with 
blood  at  the  end  of  four  months  (cf.  Fig.  12).  F.  No  trace  left  after 
three  and  a  half  months.  0.  Slight  traces  still  present  after  three 
months.  H.  Completely  gone  in  two  months. 

Most  haemorrhages  are  of  homogeneous  red  colour,  but  often 
in  leukaemia  and  sometimes  in  other  conditions  some  have  a 
pale  nucleus-like  centre  ;  large  dense  haemorrhages  are  often 


DISAPPEARANCE  37 

dark  and  have  a  slaty  hue  over  their  central  parts,  and  many 
large  single  haemorrhages  have  a  mottled  red  and  white  appear- 
ance (Figs.  12  and  13;  E). 

All  haemorrhages  tend  to  become  absorbed,  but  in  many 
diseases  new  ones  develop  as  rapidly  as  the  old  ones  clear,  so 
that  the  retina  is  never  free  of  their  presence. 

In  the  great  majority  of  cases,  haemorrhages  as  seen  ophthal- 
moscopically  gradually  become  smaller  and  simply  fade  away 
without  undergoing  change  of  colour,  till  ultimately  no  trace  of 
their  former  presence  is  left. 

This  is  also  true  of  large  haemorrhages  (Figs.  12  and  13,  E) ; 
if,  however,  the  external,  limiting  membrane  is  transgressed, 
pigmentary  disturbance  will  be  evident  after  the  blood  is 
absorbed. 

As  would  be  expected,  the  time  taken  for  the  absorption  of 
haemorrhages  varies  with  their  size  and  density. 

In  Fig.  13,  individual  haemorrhages  which  disappeared  under 
observation  have  been  incorporated  in  one  composite  figure,  in 
which  the  size  and  texture  of  the  haemorrhages  has  been  indicated 
approximately,  and  the  time  of  complete  disappearance  noted. 

Apart  from  well-recognised  causes,  retinal  haemorrhages 
occur  in  what  seems  to  be  an  accidental  sort  of  way ;  for 
example,  in  patients  who  are  apparently  quite  healthy ;  or  a 
patient  who  is  emaciated,  or  seriously  ill  from  any  cause,  may 
be  found  to  have  developed  one  or  two  small  haemorrhages 
into  the  retinal  tissues. 

THE   HEMIANOPIC   PUPIL   REACTION. 

This  reaction  often  goes  by  the  name  of  Wernicke's  pupil 
reaction.  The  term  implies  that  in  cases  of  hemianopia,  upon 
throwing  light  on  to  that  half  of  the  retina  which  corresponds  to 
the  blind  half  of  the  field  of  vision,  the  pupil  does  not  contract, 
whereas  it  does  contract  when  light  reaches  the  opposite  half 
of  the  retina. 

The  test  is  supposed  to  be  of  value  in  distinguishing  a  hemi- 
anopia due  to  a  lesion  in  front  of  the  mid-brain,  from  one  in 
which  the  lesion  is  posterior  to  the  mid-brain. 


38  HEMIANOPIC  PUPIL  REACTION 

Experimental  investigations  upon  the  area  of  the  retina 
which,  on  stimulation  by  light  will  cause  contraction  of  the 
pupil,  are  not  altogether  in  accord  ;  the  difficulty  arises  from 
the  impossibility  of  stimulating  one  localised  spot  without  the 
rest  of  the  retina  being  simultaneously  stimulated  by  diffusion 
within  the  eye. 

Hess  1  investigated  the  matter  by  alternately  impinging  a 
beam  of  light  on  the  optic  disc,  and  then  on  other  parts  of  the 
retina  which  were  equidistant  with  it  from  the  yellow  spot. 

In  all  cases  the  diffusion  of  light  within  the  eye  was  the  same, 
but  in  the  first  instance  no  light  percipient  elements  were 
directly  stimulated,  yet  the  pupil  contraction  was  the  same  as 
when  light  was  thrown  on  to  parts  of  the  retina  in  which  light 
percipient  elements  were  present ;  the  deduction  is  that  the 
pupil  contraction  in  all  cases  was  due  to  intra-ocular  diffusion 
of  light  stimulating  the  yellow  spot  and  its  immediate  vicinity. 

Hess  comes  to  the  conclusion  that  no  pupil  contraction  could 
be  called  forth  by  his  method  outside  a  circle  of  3  mm.  radius 
from  the  macula. 

These  conclusions  were  in  part  confirmed  by  Hesse.2 

Hesse  indeed  succeeded  in  showing  that  with  a  powerful  light 
the  pupil  could  be  made  to  contract  by  stimulating  the  retina 
/  at  45  degrees  from  the  yellow  spot  ;  he  concludes  that  whilst 
he  has  shown  that  the  hemianopic  pupil  phenomenon  is  theoreti- 
cally possible,  it  has  no  practical  application. 

Henschen  3  believes  it  is  of  value,  and  analyses  the  precise 
site  of  the  lesions  which  may  produce  it. 

Best  4  from  clinical  considerations,  adds  weight  to  the  view 
that  the  pupillomotor  and  visual  functions  of  the  retina  are 
co-terminous. 

He  reports  a  case  in  which  with  only  a  small  peripheral 

island  of  visual  field  left,  the  pupil  contracted  well  to  light,  and 

points  out  how  close  is  the  parallelism  between  the  brightness 

.  of  light  and  the  pupil  reaction  in  disease  of  the  optic  nerve  and 

retina. 

1  Archiv.  f.  Augen.,  1907,  Iviii.,  182. 

2  Klin.  Monats.f.  Aug.,   1909,  xlvii.,  33. 

3  Klin,  und  Anat.  Beitrdge  zur  Pathol.  des  Gehirns,  Theil,  iii. 

4  Archiv.  f.  Augen,  1908,  Lxi ,  319. 


ITS    VALUE  39 

Thus  he  states,  with  retro -ocular  neuritis,  he  has  seen  blind- 
ness associated  with  impaired  pupillary  reactions,  and  with  the 
gradual  return  of  vision  there  has  always  been  a  corresponding 
pupil  reaction. 

He  concludes  that  it  cannot  at  present  be  decided  from 
physiological  or  pathological  investigation  whether  there  are 
special  pupillomotor  fibres  or  not. 

/ 1  have  many  times  applied  Wernicke's  test  in  cases  of  chias- 
mal  lesions,  but  have  never  found  a  convincing  result,  or  reaped 
any  advantage  from  it.  I  have  not  used  the  elaborate  apparatus 
of  the  physiological  investigators. 

/Ai  the  present  time  it  may  be  concluded  with  Hesse  that 
whilst  the  hemianopic  pupil  phenomenon  is  theoretically 
possible,  it  has  no  practical  application,  and  on  account  of  the 
fallacies  which  attach  to  earlier  methods,  the  results  hitherto 
reported  are  of  no  value/. 

BI-NASAL   HEMIANOPIA. 

The  occurrence  of  visual  fields  of  such  kind  as  to  justify  the 
term  bi-nasal  hemianopia  is  unusual,  and  when  it  occurs  the 
evidence  goes  to  show  that  it  is  always  due  to  a  lesion  at  or  in 
front  of  the  chiasma,  for  most'  have  been  cases  in  which  papill- 
cedema  or  a  consecutive  nerve  atrophy  has  been  present,  and 
intone  has  there  been  a  sharply  divided  field  at  all  comparable 
with  that  seen  in  cortical  lesions. 

The  possibility  of  an  anatomical  variation  in  the  crossing  of 
the  fibres  may  be  had  in  mind,  for  should  such  variation  take 
the  form  of  a  complete  decussation,  as  occurs  in  some  animals, 
a  pituitary  tumour  would  be  likely  to  result  in  bi-nasal  rather 
than  in  bi-temporal  defects. 

Frequency.— The  condition  is  not  so  rare  as  might  be  sup- 
posed, for  Gushing  and  Walker,1  amongst  300  cases  of  intra- 
cranial  growths,  found  nineteen  which  exhibited  a  tendency 
towards  bi-nasal  hemianopia  ;  they  state  "  it  occurs  as  a  late 
sequel  of  an  advanced  choked  disc  in  the  stage  of  recession  of 
the  oedema,  and  a  bilaterally  symmetrical  process,  implying 

1  Archiv.  of  Ophth.,  1912,  xli.,  597. 


40  BI-NASAL  HEMIANOPIA 

an  equal  degree  of  involvement  of  the  two  eyes,  suggests  as  a 
rule  a  distant,  often  a  cerebellar  lesion  with  secondary  hydro- 
cephalus. 

Veasey  l  also  has  collected  twenty  cases. 

The  mechanism  of  production  is  attributed  to  pressure  of  the 
carotid  vessels  upon  the  outer  aspect  of  the  chiasma,  either 
because  the  chiasma  is  forced  outwards  against  the  artery,  or 
because  the  artery,  being  diseased,  presses  upon  the  chiasma  ; 
thus  Gushing  and  Walker  say  "  an  internal  hydrocephalus  with 
distension  of  the  third  -ventricle,  crowds  the  optic  nerves 
downward  and  outward  against  the  carotid  vessels  which 
transversely  indent  the  outer  aspect  of  the  optic  nerves." 

Knapp  2  suggests  conversely,  that  the  outer  aspect  of  the 
nerves  may  be  pressed  upon  by  markedly  diseased  internal 
carotid  or  posterior  communicating  arteries,  and  nasal  hemia- 
nopia  or  partial  optic  atrophy  be  so  produced. 

Heed  and  Price  3  state  that^of  twenty-one  recorded  cases  the 
lesion  could  be  located  in  the  optic  nerve  in  fifteen.  They 
report  a  case  occurring  in  tabes,  and  similar  cases  are  reported 
by  Fuchs,  and  Lang  and  Beevor.V 


THE  RETENTION  OF  THE  PUPILLARY  LIGHT  REFLEX 
IN   CASES   OF   BLINDNESS. 

There  are  a  few  conditions  in  which  the  pupil  will  contract 
to  light  stimulus  even  though  the  patient  is  sightless. 

The  most  important  examples  of  this  are  seen  in  ursemic 
blindness  (p.  158)  ;  the  amaurosis  of  post-baslc~~memngitis 
(p.  203)  ;  and.  puerperal  amaurosis  (p.  138)— conditions  which 
are  believed  to  be  due  to  the  effects  of  toxins  on  the  visual 
cortex. 

Edmunds  5  reports  the  case  of  ItfTidiot,  aged  two  and  a  half 
years,  who  was  said  to  be  totally  blind,  in  whom  the  fundi  were 
normal  and  the  pupils  reacted  well  to  light. 

1  Ophth.  Record,  1897,  78. 

2  Arch.  Scient.  and  Pract.  MedJand  Surg.,  1873,  293. 

3  Jour.  Amer.  Med.  Ass.,  1914'Jlxii.,  771. 

4  Trans.  Ophth.  Soc.  U.K.,  1894  xiv    248 
6  Path.  Trans.,  1881,  xxxii.,  4. 


INDIRECT  PARALYSIS  OF  NERVES  41 

On  post-mortem  examination  the  cerebral  hemispheres  and 
optic  thalami  were  very  small,  and  from  an  examination  of  the 
specimen  in  the  museum  of  St.  Thomas'  Hospital,  Nettleship 
says  :  "I  find  the  corpora  quadrigemina  are  quite  healthy- 
looking  and  plump,  contrasting  most  markedly  with  the 
adjacent  optic  thalami,"  a  point  which  lends  weight  to  the  view 
that  the  corpora  quadrigemina  are  not  concerned  with  the 
actual  act  of  seeing  (p.  108)  ;  it  is  of  interest,  however,  to 
remember  that  in  animals  in  which  it  would  seem  that  sight  is 
of  preponderating  importance,  such  as  birds  and  reptiles,  the 
superior  corpora  are  particularly  well  developed,  suggesting 
that  they  are  concerned  in  an  important  way  with  the  functions 
of  sight. 

Cases  of  optic  atrophy  from  which  the  patients  were  blind, 
but  in  which  the  pupils  dilated  in  the  dark  and  contracted  in 
sunlight,  are  reported  by  Harlan  1  and  Myles  Standish  and 
de  Schweinitz. 

INDIRECT   PARALYSIS   OF   CRANIAL  NERVES. 

It  is  well  recognised  that  intra-cranial  lesions,  be  they  tumours, 
inflammations,  or  some  less  tangible  form  of  disease,  are  not 
infrequently  associated  with__]jaralysis  of  cranial  nerves  with 
which  no  obVious  directl*elationship  exists  ;  the  vulnerability 
of  the  sixth:  nerve  in  this  respect  is  notorious. 

Thus  tumours  in  any  part  of  the  cerebrum,  where  it  seems  any 
direct  connection  with  the  sixth  or  third  nerve  can  be  excluded, 
may,  nevertheless,  be  accompanied  by  paralysis  of  one  or  both 
of  these  nerves  ;  they,  too,  are  often  involved  in.  cases  of 
meningitis,  where  j)ost-mortem  there  seems  to  be  no  sufficient 
reason  for  their  selective  implication,  and  the  picking  out  of  the 
sixth  nerve  in  cases  of  spinal  anaesthesia  seems  to  call  for  special 
elucidation. 

Harvey  Gushing  2  says  :  "  It  is  a  matter  of  common  knowledge 
that  ocular  palsies,  either  stationary  or  fluctuating  in  their 
intensity,  particularly  those  manifested  by  a  convergent  squint, 
are  so  often  misleading  from  the  standpoint  of  topographical 

1  Trans.  Amer.  Ophth.  Soc.,vii.,  672. 

2  Brain,  1910,  xxxiii.,  204. 


42  INDIRECT  PARALYSIS  OF  NERVES 

diagnosis,  that  they  have  come  to  be  classed  with  the  "  false 
localising  signs  "  of  tumour. 

Thus,  James  Collier  1  found  that  false  localising  signs  attri- 
butable to  the  "  indirect  intra-cranial  results  of  intra-cranial 
new  growth  "  were  present  in  12-5  per  cent,  of  his  161  cases. 

He  emphasises  the  fact  that  the  sixth  nerve  is  much  more 
frequently  implicated  in  this  way,  and  that  it  was  affected  in 
twelve  of  his  cases  in  which  the  tumour  was  situated  above  the 
tentoriunis 

Paralysis  of  cranial  nerves,  and  especially  of  the  abducent, 
has  resulted  in  quite  a  number  of  cases  from  spinallinsesthesia. 
It  is  usually  a  paresis  only,  it  comes  on  within  an  hour  or  two 
of  the  administration,  and  is  usually  slowly  recovered  from  in 
the  course  of  many  weeks.  Reber 2  reports  five  such  cases 
amongst  2,000  spinal  anaesthetics,  in  nearly  all  of  which  stovaine 
was  used.  He  collects  thirty^six  cases  which  have  been  reported, 
and  in  all  but  four  of  these  it  was  the  external  rectus  muscle 
alone  which  was  paralysed. 

Explanations. — Several  theories  have  been  propounded  to 
explain  these  lesions,  the  oldest  and  least  satisfying  attributing 
the  vulnerability  of  the  sixth  nerve  to  its  long  intra-cranial 
course. 

Collier  says  :  "If  the  paralysis  of  these  nerves  is  the  result 
of  shifting  backwards  of  the  brain  stem  from  supra-tcntorial 
pressure,  the  effect  being  traction  on  the  nerves  attached  to  the 
brain  stem  in  proportion  as  their  direction  is  more  nearly  a 
fronto-caudal  direction,  then  the  shifting  backwards  on  the 
brain  stem  would  cause  paralysis  of  the  sixth  nerve  first,  then 
of  the  third,  and  lastly  of  the  seventh  and  eighth,  whilst  the 
nerves  which  are  transversely  directed,  or  with  sinuous  course, 
would  be  little  affected." 

Gushing,  however,  offers  an  entirely  different  explanation. 

He  points  out  that  as  the  sixth  nerve  runs  directly  forwards 
from  the  hind  margin  of  the  pons,  it  meets  the  anterior  inferior 
cerebellar  and  internal  auditory  arteries  at  right  angles,  as  they 
course  directly  outwards  from  the  basilar  artery  (Fig.  14). 

1  Brain,  1904,  xxvii.,  490. 

*  Jour.  Amer.  Med.  Ass.,  1910,  lv.,  380. 


PATHOGENS  SIS  43 

In  many  cases  these  vessels  lie  ventral  to  the  nerve,  so  that 
the  latter  is  crossed  by  them  at  right  angles,  and  is  liable  to 
compression  between  them  and  the  pons. 

He  shows  how  acute  may  be  the  constriction  produced  in 
this  way  (Fig.  14),  and  he  believes  that  in  cerebellar  lesions 
for  instance,  the  swelling  and  enlargement  of  the  hind  brain 
may  produce  such  a  stretching  of  the  arteries  around  the  brain 


Fiu.  14. — An  antero -posterior  section  through  a  part  of  the  pons,  showing 
how  acutely  the  sixth  nerve  may  be  indented  where  it  is  crossed  at 
'right  angles  by  the  anterior  inferior  cerebellar  and  internal  auditory 
arteries,  and  so  may  have  its  functions  interrupted.  ( Harvey  Gushing. ) 

stem  as  to  constrict  the  nerves,  much  as  though  a  rubber  band 
had  been  placed  around  them  (Fig.  14). 

He  suggests  that  oedema  of  the  brain  stem  may  in  this  way 
be  responsible  for/some  cases  of  ophthalmoplegia  associated 
with  migraine,  and  for  the  paralysis  following  lumbar  ansesthesiaj 

The  third  nerve  has  a  very~dose~"felationship  with  cerebral 
vessels,  in  that  as  it  passes  forwards  from  the  sulcus  oculomo- 
torius  it  separates  the  superior  cerebellar  from  the  posterior 
cerebral  artery  ;  and  although  there  is  no  firm  structure  like  the 
pons  in  the  case  of  the  sixth  nerve,  against  which  the  nerve  can 


44  MONOCULAR  Dl PLOP 1 A 

be  compressed,  it  is  possible  that  stretching  of  these  vessels 
might  produce  a  temporary  inhibition  of  function  in  the  third 
nerve  by  its  being  nipped  between  them. 

In  favour  of  the  foregoing  explanation  it  may  be  noted,  that 
the  sixth  and  third  nerves  are  notoriously  those  which  are  most 
frequently  involved  in  indirect  paralysis,  and  it  is  just  these  two 
of  all  the  cranial  nerves  which  are  in  most  intimate  relationship 
with  large  cerebral  arteries,  and  with  arteries,  too,  which,  being 
anchored  ventrally  by  the  basilar  artery,  tend  to  encircle  the 
brain  stem,  and  are,  therefore,  most  likely  to  constrict  the  nerves 
if  swelling  of  the  brain  occurs  from  oedema.1 

MONOCULAR   DIPLOPIA. 

There  are  several  conditions  in  the  eyeball  itself  which  may 
give  rise  to  monocular  diplopia  ;  such  are  astigmatism,  opacities 
of  the  cornea  or  lens,  or,  under  certain  conditions,  a  double 
pupil. 

Such  cases  are  of  little  interest.     A  number  of  cases,  however, 

have  been  recorded  in  which  monocular  diplopia  was  com- 

I  plained  of,  where  there  was  no  local  cause  in  the  eye  to  explain 

'  it,    and   in   which,    after   careful    and    critical    consideration, 

malingering  and  hysteria  could  be  ruled  out  as  the  cause. 

It  is  worthy  of  ^special  note  that  in  most  of  these  cases  there 
has  been  a  gross  cerebral  lesion  which  has  resulted  in  some 
ocular  palsy — usually  the  sixth  nerve  or  nerves — with  its 
accompanying  binocular  diplopia,  and  that  the  monocular 
diplopia  has  developed  a  little  later  as  a  sequel  to,  or  an 
accompaniment  of,  the  binocular  diplopia. 

Such  cases  have  been  reported  by  de  Schweinitz,2  Bouveret 
and  Chapoto,3  Thompson,4  Brailey,  Juler,  Gulliver,  and  others. 

Bielschowsky  5  reports  the  case  of  a  man  who  had  a  squint, 
and  who  complained  of  diplopia  after  removal  of  one  eye. 

Three  important  cases  are  reported  by  Ord,6  Abercrombie,7 

1  Foster  Moore,  Pro.  Roy.  Soc.  Med.,  Ophth.  and  Neurol.  Sect.,  1921. 

2  Trans.  Amer.  Med.  Ass.,  Detroit,  1892,  277. 

3  Revue  de  Med.,  1892,  xii.,  728. 

4  Trans.  Amer.  Med.  Ass.,  Ophth.  Section,  1891. 
8  Ophth.  Soc.,  Heidelberg,  1897,  93. 

«  Trans.  Ophth.  Soc.  U.K.,  1882,  ii.,  201  and  205. 
'  Trans.  Ophth.  Soc.  U.K.,  1882,  ii .,  210, 


ILLUSTRATIVE  CASES  45 

and  Gunn  and  Anderson,1  in  which  gross  cerebral  disease  was 
present,  and  in  two  of  which  post-mortem  examinations  were 
made. 

In  all  of  these  one  or  both  sixth  nerves  were  involved.  In  Ord's 
case,  a  lad  of  thirteen,  both  sixth  nerves  were  affected,  there 
was  binocular  diplopia,  followed  in  a  few  days  by  monocular 
diplopia  with  either  eye,  which  later  on  became  limited  to  the 
left  eye  alone.,/  He  was  examined  by  Nettleship  from  time  to 
time,  who  states  that  the  boy  was  sincere,  but  that  there  were 
reasons  for  suspecting  that  "  his  monocular  statements  were  in 
some  way  based  on  his  experience  of  binocular  sixth-nerve 
diplopia,"  a  remark  which  would  seem  to  be  appropriate  to 
many  of  the  cases.  At  the  post-mortem  examination  a  large 
cavity  occupying  the  ordinary  position  of  the  lateral  ventricle, 
with  which  a  small  aneurysm  was  communicating,  was  found 
in  the  right  cerebral  hemisphere. 

Abercrombie's  patient  was  a  girl  of  ten  ;  she  had  paralysis 
of  the  right  external  rectus,  and  complained  of  diplopia  with 
both  eyes,  and  also  when  the  left  was  covered.  She  was 
examined  on  many  occasions,  and  always  with  the  same  result 
as  to  the  monocular  diplopia.  At  the  post-mortem  a  large 
abscess  containing  some  ounces  of  pus  was  found  in  the  right 
temporo-sphenoidal  and  occipital  lobes. 

Gunn  and  Anderson's  patient  was  a  man  of  thirty-four,  with 
many  evidences  of  gross  cerebral  disease.  He  had  paresis  of 
the  left  sixth  nerve  with  homonymous  diplopia,  and  also  left 
uniocular  diplopia  ;  in  the  latter,  as  in  the  former  case,  one 
image  being  sharp  and  distinct,  the  other  dim.  It  was  not 
found  possible  to  produce  three  images  by  means  of  a  prism. 
The  left  pupil  was  larger  than  the  right,  which  these  observers 
'/say  is  not  infrequent  in  cases  of  sixth  nerve  paralysis  ;  they  also 
comment  on  the  disappearance  of  monocular  diplopia  when 
binocular  diplopia  is  recovered  from. 

HAEMORRHAGE   INTO   THE   OPTIC  NERVE   SHEATH. 

It  will  be  remembered  that  the  supporting  layer  of  the  dura 
mater  is  carried  outwards  through  the  foramina  of  the  skull  to 
1  Trans.  Ophth.  Soc.  U.K.,  1884,  iv.,  292. 


46  BLOOD  IN   THE  OPTIC  SHEATH 

become  fused,  after  a  short  distance,  with  the  sheaths  of  the 
vessels  and  nerves  which  traverse  these  foramina. 
/  In  the  case  of  the  optic  nerve,  however,  the  dura  forms 
/a  roomy  sheath  which  extends  to  the  sclerotic,  with  which  it 
becomes  fused  around  the  optic  nerve  entrance  ;  the  pia  and 
arachnoid  are  also  continued  in  a  similar  manner. 

It  comes  about  that  the  cranial  subdural  and  subarachnoid 
spaces  are  in  free  and  direct  continuity  with  these  spaces  as 
they  surround  the  optic  nerve,  the  pial  surface  of  the  nerve  is 
freely  TVathed  by  cerebro^spinal  fluid  as  far  as  the  back  of  the 
eyeball^  and  in  this  situation  the  sheath  of  the  nerve  dilates  in 
a  bulbous  manner. 

(Reference  has  already  been  made  to  the  fact  that  the  central 
retinal  vessels,  as  they  traverse  this  space,  are  subject  to 
variations  in  the  intra-cranial  pressure  through  the  medium  of 
the  cerebro -spinal  fluid.) 

If,  then,  from  any  cause  blood  is  poured  into  the  cerebral 
subarachnoid  space,  it  is  free  to  enter  the  continuation  of  the 
space  to  the  back  of  the  globe. 

A  common  "cause  of  this  form  of  haemorrhage  is  fracture  of 
the  skull,  and  in  such  a  case,  where  blood  is  present  in  any 
quantity  in  the  anterior  cranial  fossa,  I  have  usually  found  the 
sheath  to  contain  blood,  and  sometimes  it  is  full  of  it. 

Owing  to  the  shape  of  the  space  with  a  bulbous  dilatation 
at  its  distal  end,  the  appearance  is  as  if  the  cavity  were  forcibly 
distended  ;  this,  however,  is  not  the  case,  for  there  is  no  forcing 
out  of  the  blood  when  the  nerve  is  cut  across  in  the  post-mortem 
room,  nor  is  the  apparent  distension  relieved  thereby,  though 
the  contained  blood  is  still  fluid. 

Holder,1  from  126  personally  observed  fractures  of  the  skull, 
found  that  fifty-four  had  a  fracture  of  the  optic  foramen,  and 
forty-two  of  these  had  blood  in  the  optic  sheath.  He  states 
that  he  has  never  seen  this  in  the  absence  of  a  fracture  involving 
the  optic  foramen. 

This,  however,  is  by  no  means  the  case,  for  so  long  as  there  is 
blood  free  in  the  subarachnoid  space,  it  is  able  to  flow  into  the 
nerve  sheath,  regardless  of  the  source  of  the  bleeding. 

1  "  Bericht  der  ophth.  Gesellschaft,"  Heidelberg,  1879,  9. 


SIGNS   OF  ITS  PRESENCE  47 

I  have  seen  it  occur  from  the  rupture  of  an  aneurysm  of  the 
anterior  cerebral  artery  (Fig.  15),  and  in  a  case  of  spontaneous 
meningeal  haemorrhage. 

Liebrecht 1  found  the  condition  in  55  per  cent,  of  twenty-six 
cases  of  fracture  of  the  skull. 

In  a  case  reported  by  Priestley  Smith  2  the  blood  was  entirely 
external  to  the  arachnoid,  the  subarachnoid  space  being 
distended  by  cerebro -spinal  fluid. 

In  some  cases  retinariiaemorrhages  have  been  found,  but  I 
know  of  no  statistics  as  to  their  frequency,  and^tlTere  are  no 
signs  which  enable  one  to  state  with  any  certainty  beforehand 
that    haemorrhage     into     the 
sheath  is  present^ 

The  signs  given  by  Dupuy- 
Dutemps  3  as  being  indicative 
of  it  are  : — 

Congestion  of  the  disc 
and  oedema. 

Dilatation  of  veins. 

.Retinal     haemorrhages,      FIG.  15.— A  case  in  which  blood  has 
„   •„!  i       •  „!  flowed    into    the    sub-arachnoid 

variable   in   number  and         space  of  the  optic  nerve  sheath. 

extent.  From   a  patient   who   died   from 

TT.,  the  rupture  of  an  aneurysm  of  the 

Vitreous     haemorrhages  anterior  cerebral  artery. 

rarely  ;     and     these     he 

attributes  to  obstruction  of  the  flow  in  the  retinal  vein 

where  it  traverses  the  space. 

Gonin  4  considers  that  a  moderate  degree  of  papillary  stasis, 
with  or  without  haemorrhages  along  the  retinal  vessels,  is 
suggestive  of  the  condition. 

It  is  exceedingly  doubtful  whether  the  presence  of  blood  in 
the  sheath  per  se  in  any  way  affects  vision,  nor  is  there  evidence 
of  its  organisation. 

/  Dupuy-Dutemps  3  says  that  a  unilateral  spontaneous  sheath 
haemorrhage  as  a  cause  of  sudden  blindness  has  never  been 
/verified. 

1  Archiv.f.  Augenheil,  1906,  lv.,  36. 

2  Trans.  Ophth.  Soc.  U.K.,  1884,  iv.   271. 

3  Annul.  d'Oculist,  1914,  cli.,  184. 

4  Annul.  tCOculist,  1903,  cxxix.,  cxxx.,  93. 


48  OPHTHALMOSCOPY  AT  DEATH 

When  the  optic  nerve  is  divided  as  a  result  of  a  fracture 
involving  the  anterior  cranial  fossa,  loss  of  sight  occurs  at  once, 
but  there  are  no  immediate  ophthalmoscopic  changes  in  the 
optic  nerve,  unless  the  ophthalmic  artery  is  also  divided. 

The  signs  of  optic  atrophy  begin  to  appear  at  the  end  of  about 
a  month,  and  complete  primary  atrophy  ensues. 

THE   RETINA   AT   DEATH. 

The  details  of  the  fundus  are  clearly  visible  for  a  varying 
period,  up  to  about  fifteen  minutes  after  death ;  after  this  time 
the  cornea  becomes  too  hazy  for  the  details  to  be  distinctly 
seen. 

The  whole  fundus  immediately  after  death  shows  a  very 
marked  general  pallor,  the  disc  having  the  whiteness  of  an 
advanced  optic  atrophy. 

The  retinal  arteries  are  very  rapidly  emptied  of  blood  in  a 
direction  the  reverse  of  the  normal  flow,  and  are  reduced  to 
very  fine  white  lines  which  are  scarcely  visible ;  the  veins  are 
greatly  reduced  in  calibre,  but  contain  a  column  of  blood  which 
soon  becomes  broken  up  into  sectors  of  red  cells,  separated  by 
clear  spaces,  giving  rise  to  a  beaded  appearance. 

For  a  few  minutes  after  death,  slow  movement — visible 
streaming — of  the  blood  in  the  veins  can  be  seen,  before  the 
column  becomes  broken  up,  and  shortly  afterwards  the  blood 
can  be  seen  to  flow  backwards  along  the  arteries  towards  the 
disc  until  they  are  empty. 

Usher  l  examined  six  patients  immediately  before,  during 
and  after  death. 

He  describes  the  gradual  development  of  the  beaded  appear- 
ance of  the  blood  column  in  the  veins. 

At  first,  instead  of  the  normal  homogeneous  appearance,  the 
column  of  blood  comes  to  have  a  finely  granular,  and  later  a 
more  coarsely  granular  appearance,  still  later  beading  occurs, 
the  blood  column  being  broken  up  by  clear  spaces  as  already 
stated. 

He  has  seen  the  "  visible  streaming  "  in  the  arteries  towards 
Ihe  disc  before  respiration  ceased. 

1  Ophth.  Review,  1896,  xv.,  339. 


THE  INTRA-OCULAR   TENSION  49 

The  explanation  of  these  appearances  given  by  Usher,  is  that 
the  slowing  of  the  circulation  in  the  retinal  veins  is  of  such  a 
degree,  as  to  allow  the  individual  red  blood  cells  to  become 
visible  under  the  magnification  obtained  by  direct  ophthal- 
moscopy. 

The  large  granules  being  groups  of  red  corpuscles,  the  smaller 
being  individual  cells. 

A  markedlowering  of  the  intra-ocular  pressure  may  be  present 
as  soon  as  one  minute  after  death  (Usher)  :  I  have  shown  that 
the  intra-ocular  pressure  in  normal  individuals  is  reduced  by 
'about  40  per  cent.,  within  a  few  seconds  of  occluding  the  com- 
mon carotid  artery  by  compressing  it  against  the  transverse 
process  of  the  sixth  cervical  vertebra. 

For  a  long  time  the  broken  blood  column  in  the  veins  can  be 
made  to  shift  to  and  fro  within  the  vessels  by  intermittent 
pressure  on  the  globe. 

The  diary  of  a  man  who  committed  suicide  by  suffocation 
with  charcoal  fumes  l  is  of  a  little  interest  as  indicating  the 
stage  at  which  vision  becomes  affected  in  such  a  case. 

The  subject  was  a  man  of  fifty-three,  who  wrote  down  his 
symptoms  and  the  time  at  which  they  occurred.  Thus  :— 

3.40  (First  note)  giddiness  and  pain  in  head. 

3.50  Body  becoming  numbed. 

4.10  Pain  in  head  gets  worse,  difficulty  with  breathing. 

4.30  Sight  getting  dim. 

4.40  Head  aches,  can't  see  anything. 

5.10  (Last  note)  a  domestic  request. 

Thus  consciousness  was  retained  for  forty  minutes  at  least 
after  vision  was  affected. 

\_/ 

In  executions  by  hanging,  dislocation  of  the  crystalline  lens 
of  the  eye  frequently  results. 

1  Daily  Chronicle,  December  llth,  1911. 


CHAPTER  II 

Arterio  -sclerosis — Pernicious     anaemia— Leukaemia — Chloroma — -Ohio  - 
rosis — Secondary  anaemias — Purpura — Raynaud's  disease — Serious 
loss  of  sight  from  loss  of  blood — Erythrsemia  (Vaquez's  disease) — 
Cyanosis    retinae — Retinal    haemorrhages   in   new-born    infants- 
Thrombosis  of  retinal  veins — Thrombosis  of  the  cavernous  sinus. 

ARTERIO-SCLEROSIS. 

IN  general  arterio -sclerosis  the  retinal  arteries  of  course 
share,  and  whilst  it  is  not  possible  in  all  cases  to  detect  any 
evidence  of  the  disease  with  the  ophthalmoscope,  in  the  great 
majority  it  is  possible  to  do  so  ;  consequently  arterio -sclerosis  it 
is  one  of  the  diseases  often  first  discovered  by  the  ophthal- 
mologist. 

The  retinal  vessels  can  be  examined  during  life  with  a 
minuteness  that  is  not  possible  with  any  other  vessel  in  the 
body,  for  they  are  seen  with  great  distinctness  and  under  a 
magnification  of  about  15  diameters — i.e.,  the  magnification 
obtained  by  direct  ophthalmoscopy — thus,  changes  in  their 
structure  are  readily  detected  and  the  progress  of  the  changes 
can  be  traced. 

Following  Gunn,  the  changes  which  occur  have  been  studied 
by  many  observers,  physicians  and  ophthalmologists. 

My  own  observations  are  largely  based  upon  a  batch  of 
sixty-six  patients  which  were  collected  at  the  Moorfields  Eye 
Hospital,1  all  of  whom  I  had  under  more  or  less  constant  obser- 
vation for  at  least  six  years,  or  till  the  time  of  their  death,  the 
longest  time  for  which  a  patient  was  watched  being  thirteen 
years. 

I  have  recently  reviewed  this  group  of  patients  afresh,  and 
of  them  thirty-eight  are  known  to  be  dead,  the  cause  of  death 
being  known  in  thirty -seven  ;  fourteen  have  been  re-examined, 
and  the  remaining  fourteen  I  have  been  unable  to  trace. 

1  Quarterly  Journal  of  Medicine,  1916,  x.,  29. 


OPHTHALMOSCOPIC  SIGNS  51 

The  retinal  changes  are  best  considered  under  two  heads  :— 

(1)  Retinal  arterio -sclerosis. 

(2)  Arterio -sclerotic  retinitis. 

£^, 

(1)  Retinal  Arterio-Sclerosis. — The  first^change  which  is 
observable,  but  by  no  means  the  most  reliable,  is  an  increase 
in  the  brightness  of  the  reflex  from  the  retinal  arteries,  giving 
them  a  burnished  appearance,  an  appearance  to  which  the  term 
"  copper  wire  arteries  "  is  sometimes  applied. 

The  development  of  this  appearance  is  probably  coincident 
with  the  commencement  of  thickening  of  the  middle~~coat  of 
the  artery.1 

It  is  chieflyoT'vame  when  the  branches  of  the  second  and 
third  dimensions  are  involved,  and  is  especially~significant  when 
the  central  light  streak  has  a  finely  beaded  appearance. 

This  is  a  sign  which  needs  a  more  than  usually  critical  exami- 
nation, for  in  its  initial  stages  it  is  only  an  exaggeration  of  the 
normal  appearances,  and  consequently  it  should  only  be  con- 
sidered of  importance  when  it  is  particularly  well  marked  and 
evidently  pathological. 

Arterio- Venous  Crossings. — As  the  thickening  of  the  arterial 
coat  increases  so  the  vessel  becomes  less^fansparent. 

Under  normal  conditions,  when  an  artery  crosses  a  vein,  the 
vein  is  visible  right  up  to  the  coats  of  the  artery  on  each  side, 
and  sometimes  it  may  be  seen  faintly  through  the  artery  as  it 
crosses  underneath  it. 

As  the  arterial  walls  become  thickened,  not  only  is  the  vein 
quite  invisible  where  it  lies  deep  to  the  artery,  but  also  for  some 
distance  on  each  side,  the  thickened  arterial  coats  completely 
hiding  it  from  view. 

At  a  more  advanced  stage  a  further  manifestation  is  developed 
at  these  crossings. 

At  first  the  vein  is  hidden  from  view  under  the  artery,  but 
its  course  is  not  interfered  with,  as  however  the  thickening  and 
rigidity  of  the  artery  become  increased,  the  course  of  the  under- 
lying vein  becomes  diverted. 

1  Goats,  Trans.  Ophth.  Soc.  U.K.,  1913,  xxxiii.,  37. 


52 


AETERIO-SCLEROSIS 


In  a  case  of  severe  sclerosis  the  vein,  though  meeting  the 
artery  at  an  angle  of,  say,  30  degrees  and  leaving  it  at  a  similar 
angle,  passes  under  it  at  right  angles  to  its  course  (Figs.  11 
and  19). 

The  effect  attained  is,  that  the  vein,  instead  of  taking  a  long 
oblique  course  under  the  artery,  traverses  from  one  side  to  the 
other  by  the  shortest  possible  route  ;  a  similar  phenomenon 
may  be  seen  when  a  vein  crosses  over  a  sclerosed  artery  (Figs.  11, 
16  and  19). 

When  this  phenomenon  is  seen,  it  is  safe  to  assert  that  the 
sclerosis  is  of  a  high  or  very  hfglf  degree.  I  have  watched  one 

of  these  crossings  remain  unchanged 
for  four  years,  and  whilst  it  would  seem 
that  the  flow  through  the  vein  must  be 
obstructed,  there  was  no  dilatation  of 
its  peripheral  portion,  and  no  haemor- 
rhages in  its  territory  ;  I  am  convinced 
that  any  appreciable  distension  of  the 
peripheral  portion  of  veins  in  these 
circumstances,  a  condition  to  which  the 
term  banking  is  sometimes  applied,  is 
of  quite  unusual  occurrence. 

Irregularity  of  Lumen.  —  In  some 
cases,  as  sclerosis  of  the^'artery  in- 
creases, its  lumen,  as  judged  by  the 
contained  column  of  blood,  ceases  to 
be  of  uniform  calibre  and  comes  to  show  narrowings  in  its 
course. 

These  narrowings  usually  develop  somewhat  gradually  as  the 
vessel  is  traced  along,  until  the  lumen  may  be  reduced  to 
one-halTorless  of  its  original  diameter ;  it  then  dilates  again  to 
its  former  size,  and  this  process  may  be  repeated  several  times 
in  the  same  vessel  as  it  is  traced  towards  the  periphery  (Fig.  17). 
I  have  kept  a  considerable  number  of  these  individual 
constrictions  under  observation  for  several  years,  and  have 
found  that  the  artery  and  retina  peripheral  to  them  may 
remain  quite  unaltered  for  long  periods. 

Fig.  17  represents  the  retinal  arteries  of  a  woman  seen  on 


FIG.  16. — To  show  the  riding 
of  a  vein  over  a  thickened 
artery  in  the  retina.  The 
shaded  band  on  each  side 
of  the  artery  indicates  its 
visible  coats.  From  a  man 
of  sixty-five  whose  systolic 
blood  pressure  was  265  mm. 
The  appearances  were  quite 
unchanged  at  the  end  of 
two  years. 


IRREGULARITY  OF  LUMEN 


53 


September  13th,  1913,  and  on  March  4th,  1916,  there  was  no 
discoverable  alteration  in  the  condition  of  the  vessels. 

I  have  watched  a  marked  degree  of  irregularity  of  lumen 
develop  in  eight  months  in  a  soldier  who  had  renal  retinitis 
associated  with  trench  nephritis.  The  presence  of  this  irregu- 


FIG.  17. — Showing  marked  irregularity  of  lumen  of  the  retinal  arteries  (^4) 
from  a  woman  of  fifty-seven,  whose  systolic  blood  pressure  was 
218  mm.  She  died  of  "  an  acute  heart  attack  "  four  years  and  five 
months  after  the  drawing  was  made.  At  the  end  of  two  and  a  half 
years  the  condition  was  quite  unchanged,  the  individual  constrictions 
being  unaltered.  See  charts  of  visual  fields,  Figs.  24  and  25. 

larity  of  lumen  is  always  an  indication  of  a  high  degree  of  arterial 
change. 

It  has  been  shown  by  Coats  *  that  the  above  appearances 
are  due  to  a  proliferation  of  the  endothelium  with  little  or  no 
ground  substance  between  the  cells  ;  it  is  characteristic  for  this 

1  Trans.  Ophth.  Soc.  U.K.,  1904,  xxiv.,  165 ;    1913,  xxxiii.,  30. 


54  ARTERIO-SCLEROSIS 

form  of  the  disease  to  encroach  on  the  lumen  in  an  eccentric 
manner. 

Coats  also  points  out  that  the  common  form  of  disease  in  the 
central  artery  is  confined  in  almost  all  cases  to  the  endothelium 
and  elastic  membrane  (Fig.  18),  changes  in  the  media  being 
secondary,  whilst  in  the  branches  in  the  retina  the  most  common 
form  of  disease  affects  the  media,  giving  rise  to  concentric  con- 
strictions of  lumen  and  producing  the  bright  copper  wire 
ophthalmoscopic  appearance  (Fig.  21). 


FIG.  18. — A  section  through  the  central  retinal  artery  within  the  nerve  to 
show  marked  endarteritis.     (Coats.) 

Tortuosity  of  Arteries. — When  the  walls  of  an  artery  increase 
in  thickness  the  artery  is  necessarily  increased  in  length,  for 
one  can  hardly  imagine  the  responsible  elements  increasing  in 
one  diameter  only  :  it  follows  necessarily  that  the  retinal 
arteries  are  increased  in  tortuosity  in  arterio-sclerosis ;  they, 
however,  vary  widely  in  their  tortuosity  under  normal  condi- 
tions, and  consequently  this  change,  pathological  though  it  be, 
is  by  itself  of  no-value  in  the  identification  of  retinal  arterio- 
sclerosis. 

At  times  a  very  marked  tortuosity  of  the  small  vessels  in 


HEMORRHAGES  55 

the  region  of  the  macula  is  seen,  and  when  it  occurs  it 
implies  a  marked  degree  of  arterio -sclerosis,  as  was  shown  by 
de  Schweinitz ;  it  is,  however,  of  infrequent  occurrence,  and 
consequently  it  must  be  considered  as  one  of  the  less 
important  signs. 

A  congested  appearance  of  the  disc  is  described  by 
de  Schweinitz  l  as  one  of  the  early  signs. 

Wendell  Reber  calls  attention  to  a  sluggish  pupil  reaction 
and  abnormal  recession  of  the  near  point. 

Retinal  Haemorrhages. — Retinal  haemorrhages  are  of  frequent 
occurrence  where  sclerosis  of  the  retinal  arteries  is  present. 
They  are  usually  small  and  flame-shaped,  being  in  the  nerve 
fibre  layer ;  some,  however,  occur  in  the  deeper  retinal  layers 
and  then  are  roughly  circular  in  outline.  They  are  always 
most  profuse  in  the  posterior  parts  of  the  eye  where  the  vessels 
are  biggest. 

They  are  incidental  to  the  arterial  disease  and  their  presence 
in  general  does  not  convey  any  specific  significance;  in  some 
cases,  however,  an  increase  in  their  number  precedes  the  onset 
of  serious  symptoms. 

(2)  Arterio-Sclerotic  Retinitis. — After  the  condition  of  the 
arteries  previously  described  has  been  under  progress  for  some 
years,  exudates  in  the  retinal  tissues  may  appear — i.e.,  a  con- 
dition of  retinitis  to  which  the  term  "  arterio-sclerotic  retinitis  " 
is  properly  applied,  becomes  engrafted  on  the  vascular  disease 
(Fig.  19). 

It  is  important  to  recognise,  especially  from  the  point  of  view 
of  prognosis,  that  this  retinitis  is  due  to  the  local  retinal  vascular 
disease,  and  is  quite  distinct  from  renal  retinitis,  although  the 
kidneys,  like  the  other  tissues  of  the  body,  share  in  the  general 
disease,  and  albumen  is  frequently  present  intermittently  in  the 
urine. 

The  development,  then,  of  these  retinal  exudates  is  to  be 
considered  as  indicating  a  later  stage  in  the  progress  of  the 
disease  of  the  retinal  arteries. 

The  exudate  takes  the  form  of  bright  white  dots  or  spots  in 
the  retina. 

1  Trans.  Amer.  Ophth.  Soc.,  1906,  xi.,  87. 


56  ARTERIO-SCLEROSIS 

The  individual  spots  are  seldom  of  greater  diameter  than  one 
of  the  main  veins,  but  occasionally  a  few  large  plaques  develop, 
in  which  cholesterin  crystals  may  appear  after  a  time. 


• 


FIG.  19. — A  case  of  arterio-sclerotic  retinitis.  From  a  woman  aged  sixty- 
five,  whose  systolic  blood  pressure  was  greater  than  300  mm.  She 
died  of  "  a  stroke  totally  blind  "  four  years  and  four  months  after 
the  drawing  was  made.  The  arteries  are  very  bright,  the  lower  one 
showing  a  dotted  reflex,  and  when  seen  three  years  and  one  month 
later  many  of  them  were  converted  into  fibrous  threads.  The  veins 
are  deeply  cut  into  by  the  arteries ;  in  one  place  a  vein  rides  over  a 
thickened  artery.  A  few  haemorrhages  and  a  number  of  the  di.xoiclc 
dots  of  arterio-sclerotic  retinitis  are  seen.  The  disc  edges  are 
blurred. 

The  spots  are  white,  sharp  edged,  with  no  evidence  of  oedema 
around  them,  are  roughly  circular  in  shape  and  are  seldom 
profuse  (Fig.  19). 

Sometimes  they  form  a  star  figure  around  the  macula,  or  a 


ARTERIO-SCLEROTIC  RETINITIS  57 

fan-shaped  figure  between  it  and  the  optic  disc,  at  other  times 
they  are  definitely  grouped  around  the  radicles  of  the  veins,  but 
in  most  cases  no  such  arrangement  is  discernible. 

They  are  sharp  edged  and  stable -looking,  and  are  very  slow 
to  undergo  change  ;  in  quite  a  number  of  cases,  however,  I  have 
watched  them  disappear  in  the  course  of  several  months,  and 
leave  no  trace  behind  x  (Fig.  53,  C  and  D,  p.  157),  or,  as  some 
disappear,  fresh  ones  occur,  and  by  this  means,  and  by  the 
development  of  fresh  haemorrhages  as  the  older  ones  are 
absorbed,  the  ophthalmoscopic  picture  may  preserve  its 
general  appearance  for  months  or  years. 

In  several  individual  cases  I  have  watched  the  evolution  of 
this  form  of  retinitis  from  a  case  of  uncomplicated  retinal 
arterio-sclerosis  ;  thus  I  still  have  under  observation,  i.e.,  for 
more  than  thirteen  years,  a  woman,  who  in  1908,  was  under  the 
care  of  Marcus  Gunn  and  later  under  George  Coats ;  at  first  she 
had  well-marked  signs  of  retinal  arterio-sclerosis  only  ;  there 
was  no  albumen  in  the  urine.  Three  years  later  she  had  small 
white  spots  round  the  macula  and  a  cloud  of  albumen  in  the 
urine  ;  spots  of  this  same  type  have  been  present  ever  since, 
the  urine  has  usually  contained  albumen,  and  the  blood  pres- 
sure, which  was  250  seven  years  ago,  is  now  270.  At  the 
present  time,  thirteen  years  since  she  was  under  the  care  of 
Marcus  Gunn,  she  still  has  arterio -sclerotic  retinitis,  but  is  in 
moderate  health  and  walks  to  hospital. 

Not  only  may  this  evolution  be  traced  in  individuals,  but 
amongst  my  sixty-six  patients  there  were  thirty-five  who  had 
retinal  arterio-sclerosis  only,  and  thirty-one  who,  in  addition, 
had  retinal  exudates,  i.e.,  they  had  "  arterio  -sclerotic  retinitis," 
and  an  analysis  of  these  two  groups  showed  what  one  would 
expect  to  find  were  the  latter  evolved  from  the  former,  for  the 
blood  pressure  was  higher,  the  incidence  of  cerebral  vascular 
lesions  was  more  frequent,  and  the  mortality  was  greater  in  the 
group  in  which  retinal  exudates  were  present  in  addition  to  the 
vascular  disease. 

Unilateral!  ty. — In  thirty-one  cases  of  "  arterio -sclerotic 
retinitis  "  it  was  unilateral  in  fourteen,  i.e.,  45  per  cent. 

1  Quart  Jour,  of  Med.,  1916—17,  x.,  42. 


58 


ARTERIOSCLEROSIS 


Histologically. — These  spots  are  situated  in  the  outer  mole- 
cular layer,  and  are  composed  of  hyaline-looking  material  in 
the  form  of  oval  or  circular  masses  which  stain  pink  or  purple 
in  eosin  and  haematoxylin  sections,  and  are  deeply  stained  by 
orcein  (Fig.  20). 


-B 


FIG.  20. — A  section  through  the  retina  of  a  man  whose  systolic  blood 
pressure  was  240  mm.,  and  who  died  of  cerebral  thrombosis.  In  the 
external  molecular  layer  are  three  roughly  circular  masses  of  hyaline 
exudate  deeply  stained  by  orcein ;  they  gave  rise  during  life  to  the 
appearances  of  the  white  dots  of  arterio-sclerotic  retinitis.  A.  The 
nerve  fibre  layer.  B.  The  rod  and  cone  layer. 

It  may  be  advantageous  here  to  contrast  renal  retinitis  with 
arterio-sclerotic  retinitis : — 


Renal  Retinitis. 
Due  to  disease  of  the  kidney. 


Arterio- Sclerotic  Retinitis. 

Due  to  primary  vascular  dis- 
ease. 

Ophthalmoscopic  appearances 
also  distinctive. 

Unilateral  in  45  per  cent. 

Tenure  of  life  uncertain,  but 
may  live  a  number  of  years. 

Do  not  die  in  uraemia,  but  the 
cause  of  death  is  usually 
directly  referable  to  vascu- 
lar disease. 

Other   Changes   in   the   Arteries. — As   the   arterial   changes 
become  more  and  more  advanced,  so  the  arterial  lumen  may 


Distinctive  ophthalmoscopic 

appearances. 
Always  bilateral. 
Seldom  survive  two  years. 

Frequently  die  in  uraemia. 


THE  CONDITION  OF  CEREBRAL  ARTERIES     59 

become  reduced,  until  ultimately  the  artery  is  converted  into  a 
thin  white  cord  containing  no  blood. 

Pipe-stem  Sheathing. — This  is  a  condition  in  which  a  part 
of  the  length  of  the  artery  becomes  surrounded  by  a  solid  white 
plaque  of  material  apparently  within  the  perivascular  sheath, 
giving  rise  to  a  pipe-stem  appearance. 

It  is  quite  distinct  from  the  sheathing  which  may  extend 
along  the  vessels  for  some  distance  from  the  disc. 

In  this  condition  the  arterial  lumen  is  in  no  way  interfered 
with,  and  in  spite  of  the  very  solid-looking  appearance  it  com- 
pletely disappears  in  time,  and  leaves  not  a  trace  of  its  previous 
presence. 

The  Retinal  Arteries  as  a  Guide  to  the  Condition  of  the 
Cerebral  Arteries. — Having  regard  to  the  method  of  develop- 
ment of  the  retina,  it  would  be  expected  that  the  condition  of 
its  arteries  would  be  a  close  guide  as  to  the  condition  of  the 
arteries  of  the  brain,  and  this  is  undoubtedly  the  case. 

The  matter  was  investigated  from  two  opposite  aspects.1 

On  the  one  hand  the  incidence  of  gross  vascular  cerebral 
lesions  was  discovered  in  sixty-six  patients  who  attended  the 
Moorfields  Eye  Hospital  for  symptoms  which  were  found  to  be 
dependent  upon  retinal  arterio -sclerosis,  and  on  the  other  hand 
the  condition  of  the  retinal  arteries  was  examined  in  forty-four 
patients  who  were  admitted  to  the  wards  of  St.  Bartholo- 
mew's Hospital  suffering  from  a  gross  vascular  lesion  of  the 
brain. 

As  regards  the  first  group,  the  condition  of  fifty-two  of  the 
sixty-six  patients  was  ascertained  at  the  end  of  seven  years. 

Thirty-eight  were  known  to  be  dead,  and  in  thirty-seven  of 
them  the  cause  of  death  was  discovered. 

In  seventeen  it  was  either  a  cerebral  haemorrhage  or  throm- 
bosis, and  of  the  fourteen  remaining,  there  was  satisfactory 
evidence — such  as  hemiplegia  or  monoplegia — that  six  of  them 
had  developed  vascular  lesions  of  the  brain. 

Thus  of  fifty-two  patients,  twenty-three  (44  per  cent.)  had  in 
the  course  of  seven  years  suffered  from  a  gross  vascular  cerebral 
lesion  which  in  seventeen  instances  was  fatal. 

1  Foster  Moore,  Quarterly  Jour,  of  Med.,  1916—17,  x.,  29. 


60 


ARTS&IO-8CLEROSI8 


Adams  l  from  similar  observations  found  a  percentage  of 


45. 


As  regards  the  second  group  of  forty-four  patients.  In  all 
of  them  the  diagnosis  of  the  physician  under  whose  care  they 
were,  was  either  cerebral  haemorrhage  or  thrombosis,  and  the 


1^&'rtfrfz&*& 


Fid.  21.- — Section  through  a  retinal  artery  of  a  man  aged  sixty-five,  whoso 
blood  pressure  was  240  mm.  and  who  died  of  cerebral  haemorrhage 
(see  also  Fig.  22).  During  life  his  retinal  arteries  had  an  exceedingly 
bright  and  burnished  appearance.  Fig.  22  is  from  a  section  through 
the  cerebral  arteries  of  the  same  patient. 

retinal  vessels  were  examined  after  the  use  of  a  mydriatic  with 
the  following  results  :— 


No  evidence  of 
arterio-sclerosis. 


Evidence  of  mild 

or  moderately  severe 

sclerosis. 


Evidence  of  severe 

sclerosis  including 

arterio-sclerotic 

retinitis. 


13  (30  per  cent.)     . .    12  (27  per  cent.)  . .    19  (43  per  cent.). 

Thus  of  forty -four  patients  who  were  the  subject  of  a  gross 
lesion  of  the  cerebral  arteries,  70  per  cent,  exhibited  evidence 
of  disease  in  the  retinal  arteries,  and  in  43  per  cent,  it  was  severe 
in  degree. 

Similar  observations  on  seventeen  patients  were  made  by 
Gunn,2  who  found  definite  evidence  of  disease  of  the  retinal 
arteries  in  59  per  cent. 

The  close  relationship  which  exists  between  disease  of  the 
retinal  and  of  the  cerebral  arteries,  then,  is  clear.  It  is  prob- 
ably true  that  where  disease  of  the  retinal  arteries  is  patent  to 
ophthalmoscopic  examination,  disease  of  the  cerebral  arteries 

1  Brit.  Jour,  of  Ophth.,  1920,  iv.,  310. 

*  Trans.  Ophth.  Soc.  U.K.,  1898,  xviii.,  376. 


ATHEROMA  61 

is  certainly  present,  but  that  in  a  certain  number  of  cases — 
30  per  cent,  according  to  the  foregoing  investigations — even 
advanced  disease  of  the  cerebral  vessels  may  be  present,  though 
the  retinal  arteries  reveal  no  evident  disease  to  the  ophthal- 
moscope. 

Sclerosis  of  the  choroidal  vessels  gives  no  information  as  to 
the  condition  of  the  cerebral  vessels.1 

Atheroma  of  the  great  Vessels. — With  a  view  to  determining 
whether  there  were  any  vascular  or  other  changes  of  the  eye 
associated  with  atheroma  of  the  great  vessels,  the  fundi  of 


FIG.  22. — Section  of  one  of  the  arteries  of  the  anterior  perforated  space  of 
a  man  aged  sixty-five,  whose  systolic  blood  pressure  was  240  mm. 
and  who  died  of  a  cerebral  haemorrhage.    Fig.  21  shows  a  section  of  a 
branch  of  the  retinal  artery  from  the  same  patient. 
\S 

twenty-one  patients,  all  of  whom  were  males  suffering  from 
intra-thoracic  aneurysms,  were  examined. 

The  age  of  the  men  varied  between  thirty-six  and  sixty-one  ; 
the  average  systolic  blood  pressure  was  141  mm. 

There  was  no  evidence  of  any  considerable  disease  of  the 
retinal  arteries  in  any  case. 

In  four  it  was  noted  that  the  veins  were  a  little  full,  and  in 

1  Ceis,  Klin.  Monats.f.  Aug.,  1911,  Ixix.  14. 


62 


AETERIO-SCLEROSIS 


one  the  arteries  are  marked  as  being  a  little  bright,  but  apart 
from  these  the  vessels  are  all  noted  as  being  quite  normal. 

In  three  cases  there  was  evidence  of  past  syphilitic  disease  of 
the  eye. 

Prognosis. — The  tenure  of  life  of  patients  who  are  the  subject 
of  retinal  arterio -sclerosis  is  uncertain,  but  a  considerable 
number  of  them  live  for  several  or  many  years. 

Retinal   haemorrhages   are   incidental   to   the   local   retinal 


FIG.  23.— A  section  through  the  retina  and  choroid  to  show  a  mild  degree 
of  degeneration  in  the  choroidal  arteries.     A.  Choroid.   B.  Retina. 


vascular  disease,  and  unless  they  are  profuse  do  not  add  gravity 
to  the  prognosis,  except  in  so  far  as  they  are  more  frequent  in 
the  more  severe  cases. 

On  the  other  hand,  as  has  been  pointed  out,  arterio -sclerotic 
retinitis  is  to  be  looked  upon  as  an  indication  of  a  further 
development  of  the  vascular  disease,  and  consequently  its 
presence  does  imply  a  more  serious  prospect,  for  of  thirty-five 
patients  with  retinal  arterio -sclerosis,  eight  were  known  to  have 
died,  and  nineteen  were  known  to  be  alive,  and  after  the  same 


AMAUROSIS  FUGAX  63 

• 

interval,  of  thirty-one  patients  with  arterio -sclerotic  retinitis, 
sixteen  were  known  to  have  died  and  twelve  were  known  to  be 
alive. 

The  prognosis  of  this  latter,  however,  differs  markedly  from 
that  implied  by  the  presence  of  renal  retinitis,  for  of  seventeen 
women,  ten  were  known  to  be  alive  for  an  average  of  three 
years  and  five  months  after  the  onset  of  symptoms,  and  of 
eleven  males,  two  are  known  to  be  alive  for  an  average  of  two 
years  and  ten  months  after  the  onset  of  symptoms,  periods 
which  much  exceed  the  length  of  survival  after  the  discovery 
of  renal  retinitis. 

As  regards  retinal  arterio -sclerosis  in  general,  Adams  con- 
cludes that  the  prognosis  is  dependent  upon  the  associated  renal 
and  cardiac  changes,  that  it  is  aggravated  by  the  presence  of 
albummuria,  and  that  it  is  better  in  older  patients. 

Sex  Incidence. — If  one  judges  from  the  eye  manifestations  of 
arterio-sclerosis,  the  condition  is  certainly  more  common  in 
women,  and  in  them  the  highest  degrees  are  seen — they  seem  to 
be  more  tolerant  of  high  blood  pressure  than  men. 

Of  sixty-six  patients  there  were  ten  whose  systolic  blood 
pressure  was  higher  than  250,  and  of  them  seven  were  women. 

In  the  case  of  one  woman  the  blood  pressure  taken  each 
month  for  six  months  was  greater  than  300  mm.  on  five  of  these 
occasions.  She  lived  for  four  years  and  four  months  after  the 
time  she  came  under  observation,  and  died  of  "  a  stroke." 

The  observations  of  Gunn,  Adams  and  Nettleship  are  all  in 
accord  with  the  above. 

The  Complications  of  Sclerosis  of  the  Retinal  Arteries. — 
Amaurosis  Fugax  may  be  conveniently  considered  here. 

This  term  is  applied  £o  paroxysmal  attacks  of  blindness, 
which  usually  affect  one'eye  only  at  a  time,  and  are  followed  by- 
recovery  of  vision  in  periods  varying  from  a  few  minutes  to 
several  hoursj  In  most  cases  it  is  ascribable  to  local  arterio- 
sclerosis, but  this  may  produce  the  effect  in  more  than  one  way. 
In  some  cases  it  would  seem  that  spasm  of  the  artery  occurs  ; 
the  nutrition  of  the  retina  is  temporarily  interrupted,  with 
consequent  loss  of  its  function  ;  recovery  of  vision  follows  as 
relaxation  of  the  spasm  takes  place. 


64 


ARTERIOSCLEROSIS 


Noyes  1  says  :  "In  consequence  of  vaso-motor  irritation  the 
retinal  artery  may  be  firmly  contracted  and  arrest  completely 
the  circulation."  Hughlings  Jackson  observed  spasm  of  the 
arteries  during  an  epileptic'fit.  Parisotti  2  saw  constriction  of 
the  upper  retinal  artery,  with  corresponding  loss  of  the  lower 
part  of  the  visual  field  in  a  case  of  ophthalmic  ^migraine. 


FIG.  24. — To  show  the  entirely  irregular  nature  of  the  defects  in  the 
visual  fields  resulting  from  severe  sclerosis  of  the  retinal  arteries. 
From  a  woman  of  fifty-seven  whose  systolic  blood  pressure  was  218 
mm.,  and  who  died  four  years  and  five  months  later  from  an  "  acute 
heart  attack."  For  condition  of  the  arteries  see  Fig.  17. 

Farabelli  3  reports  a  case  due  to  the  presence  of  a  tape  worm 
in  the  intestine.  Greenwood  4  believes  that  spasm  occurs  most 
often  in  the  early  stage  of  arterio -sclerosis. 

Sachs  describes  5  peristaltic^TTEef  constrictions  which  followed 


1  "  Diseases  of  the  Eye,"  1890,  546. 

2  Ann.  cCOcul,  cxix.,  323. 

3  Ann.  di  Ottal.,  xvi.,  63. 

4  Jour.  Amer.  Med.  Ass.,  1905,  xliv.,  781. 

5  Beitrdge  zur  Augenheilk.,  1901,  xliv.,  323. 


ARTERIAL  SPASM 


65 


each  other  along  the  course  of  the  artery.  Wagemann  1 
examined  the  retinal  vessels  of  a  man  of  sixty -nine  who  was 
the  subject  of  arterio-sclerosis  during  an  attack  of  loss  of  sight. 
He  was  quite  blind  and  the  arteries  were  reduced  to  fine 
threads.  In  thirty  minutes  the  arteries  were  again  well  filled 
and  vision  was  restored  ;  Langdon  2  reports  a  somewhat  similar 
case. 

It  would  seem  then  that  the  occurrence  of  spasm  is  substan- 


tiated although  it  is  seldom  seen,  and  as  has  been  pointed  out 
(vide  supra),  localised  constrictions  in  the  arteries,  which  are  due 
to  organic  change — for  they  may  remain  quite  unaltered  for  a 
number  of  years — may  strongly  suggest  a  localised  spasm  and 
are  often  associated  with  damaged  sight. 

In  other  cases  loss  of  sight  is  more  directly  attributable  to 
sclerosis  of  the  arteries. 

It  has  to  be  remembered  that  a  systolic  blood  pressure  of, 

1  Graefe's  Archiv.  f.  Ophth.,  1897,  xliv.,  219. 

2  Trans.  Amer.  Ophth.  Soc.,  1912,  xiii.,  797. 


66  ARTERIO-SCLEROSIS 

/say,  250  mm.  in  the  brachial  artery,  does  not  necessarily  imply 
that  the  pressure  in  the  retinal  artery  is  above  the  normal,  and 
I  have  pointed  out, :  indeed,  that  there  is  little  doubt  that  in 
many,  perhaps  all  cases  of  severe  arterio-sclerosis,  the  pressure 
in  vessels  of  the  size  of  the  retinal  arteries  is  actually  less  than 
the  normal,  a  view  which  is  confirmed  by  Priestley  Smith. 

This  being  so,  the  blood  supply  of  the  tissues  is  precarious  and 
is  easily  disturbed,  so  that  a  drop  in  the  general  blood  pressure 
from  any  cause,  may  result  in  the  supply  of  blood  to  these 
tissues  falling  below  what  is  essential  to  the  continuance  of 
their  functions,  and  whilst  such  a  happening  in  many  organs 
might  cause  little  observable  effect  unless  prolonged,  if  the  eye 
is  involved  the  loss  of  sight  cannot  be  overlooked. 

Three  chief  results  may  now  follow  ;  if  the  blood  pressure  is 
again  restored  the  sight  may  be  completely  recovered  ;  if  the 
ischsemia  has  been  prolonged  the  retinal  tissues  may  be  so 
damaged  that  recovery  does  not  take  place  ;  and,  thirdly,  the 
vessel  wall  being  diseased,  thrombosis  of  the  artery  with 
permanent  loss  of  sight  may  result. 

Perhaps  some  cases  of  Amaurosis  Fugax  may  be  compared 
with  intermittent  dafidication,  the  retinal  arteries,  owing  to 
their  diseased  condition,  being  incapable  of  providing  an 
increased  supply  of  blood  to  the  retina  when  the  demands  of 
increased  function  call  for  it. 

Thrombosis  of  the  artery  is  of  by  no  means  rare  occurrence, 
and  there  is  no  doubt  that  many  cases  of  so-called  embolism 
of  the  central  retinal  artery  are  really  of  this  nature. 

James  Taylor  2  says  in  this  connection  :  "I  have  been  struck 
with  the  fact  that  in  many  cases  I  have  seen  it  was  impossible 
to  find  any  probable  source  from  which  an  embotus  could 
I/  start." 

The  chief  symptom  is,  the  sudden  painless  loss  of  sight  in  one 
eye  in  a  patient  the  subject  of  arterio-sclerosis  ;  the  average 
blood  pressure  in  nine  patients  in  whom  this  happened  was 
225  mm.  ;  the  blindness  is  permanent  and  essentially-complete. 

Within  a  very  short  time,  opacity  of  the  central  parts  of  the 

1  Trans.  Ophth.  Soc.  U.K.,  1916,  xxxvi.,  319. 

2  Trans.  Ophth.  Soc.  U.K.,  1913,  xxxiii.,  3. 


OPTIC  ATROPHY  67 

retina  develops,  due  to  coagulation  necrosis  of  its  tissues,  and 
the  macula  shows  up  by  contrast  as  a  bright  cherry  red  spot. 

These  changes  have  been  seen  to  develop  by  de  Schweinitz  x 
within  ten  minutes  of  the  occurrence  of  the  stoppage  of  the 
blood  supply. 

In  addition,  the  blood  column  in  the  vessels  is  broken  up  into 
small  blocks  of  red  cells  with  intervening  clear  spaces  of  plasma, 
a  characteristic  picture  to  which  the  term  "  cattle  truck 
appearance  "  is  applied  ;  by  this  means  it  can  be  seen  clearly 
that  the  circulation  in  the  vessels  is  slow  or  intermittent. 

In  the  course  of  about  seven  days  the  central  parts  of  the 
retina  again  become  clear  and  the  cherry  spot  at  the  macula 
disappears. 

Later  'on  the  optic  disc  undergoes  atrophy  and  the  retinal 
vessels  become  permanently  reduced  in  size. 

Optic  Atrophy. — In  cases  of  marked  arterio-sclerosis  the 
retinal  blood  supply  may  be  gradually  so  reduced  as  a  result  of 
the  local  arterial  disease  that  it  is  no  longer  sufficient  for  the 
needs  of  the  retina. 

In  this  case  a  gradual  atrophy  of  the  retina  and  optic  nerve 
takes  place  with  a  gradual  loss  of  sight. 

A  cherry -red  spot  does  not  occur,  but  a  low  degree  of  oedema 
of  the  nerve  head  may  sometimes  be  seen  and  it  may  precede 
the  loss  of  vision. 

The  field  of  vision  is  constricted  in  an  entirely  irregular 
manner,  corresponding  with  the  varying  degree  of  involvement 
of  the  branches  of  the  retinal  artery  (Figs.  24  and  25). 

Of  eight  patients  in  whom  this  form  of  optic  atrophy  occurred, 
one  only  had  a  blood  pressure  of  less  than  200  mm.,  and  the 
average  for  the  eight  was  227  mm.2 

Otto  Reinhard  and  Pechin  and  Rollin  3  have  each  described 
atrophy  of  the  optic  nerve  from  the  direct  pressure  of  a  sclerosed 
internal  carotid  or  ophthalmic  artery  (see  also  p.  40). 

Glaucoma. — It  is  sometimes  assumed  that  glaucoma,  a 
disease  which  is  characterised  by  an  increase  of  the  intra- 

1  Trans.  Amer.  Ophth.,  1912—14,  xiii.,  815. 

2  Quart.  Jour,  of  Med.,  1917,  x.,  37. 

3  Archiv.  cTOphthal.,  1903,  xxiii.,  577. 

5— a 


68  ARTERIO-SCLEROSIS 

ocular  pressure,  is  dependent  upon  an  increase  of  blood  pressure  ; 
that,  however,  there  is  any  connection  between  the  two  is 
denied  by  so  high  an  authority  as  Priestley  Smith. 

The  intra-ocular  pressure  is,  of  course,  dependent  upon  the 
maintenance  of  the  blood  pressure,  and  it  is  quite  easy  to 
demonstrate,  by  pressure  upon  the  carotid  artery  in  man,  that 
the  pressure  inside  the  eye  can  be  temporarily  reduced  by  this 
means  by  about  40  per  cent.,1  but  it  has  been  made  clear  that 
there  is  no  relation  between  a  high  systolic  pressure  in  the 
brachial  artery  and  glaucoma. 

Thus  the  blood  pressure  of  fifty  patients  suffering  from 
primary  glaucoma,  whose  average  age  was  fifty-six,  was 
157  mm.,  which  cannot  be  considered  excessive  for  patients  of 
this  age. 

Conversely  the  average  intra-ocular  pressure  of  thirty-seven 
patients,  all  of  whose  blood  pressures  were  less  than  160  mm., 
was  14-3  mm.,  whereas  that  of  twenty -six  patients  whose  blood 
pressure  was  in  every  case  greater  than  220  mm.,  was  15-3  mm.  ; 
that  is,  in  the  latter  group  the  intra-ocular  pressure  was  1  mm. 
higher  only,  although  the  average  blood  pressure  was  higher  by 
78  mm.2 

In  a  good  many  cases  of  thrombosis  of  the  central  retinal 

vein,  and  in  a  few  cases  of  renal  and  diabetic  retinitis,  glaucoma 

supervenes,  and  in  so  far  as  these  conditions  are  associated  with 

arterio-sclerosis  the  latter  may  be  considered  to  have  an  indirect 

casual  relationship  with  glaucoma ;   apart  from  this  there  is 

lone  general  disease   only   with   which    glaucoma   is   directly 

]  associated. 

A  disease  of  an  epidemic  nature  occurred  in  the  region  of 
Calcutta  which,  whilst  a  good  deal  like  beri  beri,  was  distinct 
from  it ;  it  was  termed  epidemicdropsy,  and  it  is  a  point  of 
some  interest  that  a  considerable  proportion  of  the  patients 
affected,  developed  chronic  glaucoma,  which  seems  to  have  been 
especially  frequent  in  the  1909  epidemic.3 

1  Foster  Moore,  Roy.  Lond.  Ophlh.  Hosp.  Reports,  1915,  xx.,  117. 

2  Foster  Moore,  Roy.  Lort,d.  Ophth.  Hosp.  Reports,  xx.,  115. 

8  Mitra,  Cakutta  Med.  Journal,  1910,  41.  Ghosh,  Calcutta  Med  Journal,  1910, 
121. 


69 


PERNICIOUS   ANAEMIA. 

It  has  been  pointed  out  that  in  any  severe  anaemia,  be  its 
cause  what  it  may,  retinal  haemorrhages  may  occur  ;  in  perni- 
cious anaemia  it  is  unusual  to  find  them  absent. 

In  addition,  there  are  other  fundus  changes  of  less  conspicuous 
aspect,  which  are  not  distinctive.  Of  nineteen  cases  which  I 
had  under  observation  for  periods  up  to  three  years,  four  were 
females  and  fifteen  were  males ;  the  average  age  of  the  former 
was  forty-five  and  of  the  latter  forty -four. 

The  average  red  blood  count  was  1,223,800,  the  lowest  being 
530,000  ;  the  average  percentage  of  haemoglobin  was  32-5,  the 
lowest  being  16  ;  the  average  colour  index  was  1-31,  the  lowest 
being  0-7. 

Of  these  nineteen  cases  retinal  haemorrhages  were  found  in 
all  but  three  ;  in  some  instances  care  was  required  for  their 
discovery. 

They  are  mostly  small  and  flame-shaped,  or  roughly  circular 
in  outline,  but  larger  ones  may  develop,  and  at  times  the  retina 
becomes  widely  infiltrated  with  blood ;  *  they  are  usually 
situated  in  the  nerve  fibre  layer  and  amongst  the  ganglion  cells, 
and  occur  only  in  the  posterior  part  of  the  eye.2 

Haemorrhages  having  pale  centres  are  not  infrequent  and 
small  white,  rather  woolly-looking  areas  are  sometimes  seen 
(see  also  p.  77). 

The  retinal  vessels  are  seldom  reduced  in  size,  but  more  often 
are  somewhat  engorged  ;  this  is  occasionally  rather  marked. 

The  fundus  may  show  considerable  pallor,  but  this  varies 
much,  and  there  is  often  disparity  between  the  degree  of  the 
anaemia  and  the  pallor  or  otherwise  of  the  fundus. 

The  optic  disc  is  often  pale ;  it  was  noted  as  being  conspi- 
cuously so  in  seven  out  of  the  nineteen  cases.  It  is  probably  only 
an  expression  of  the  general  anaemia  in  most  instances,  but  at 
times  may  represent  an  actual  partial  atrophy  of  the  nerve  con- 
sequent upon  the  areas  of  degeneration  in  the  retina. 

It  is  common  to  find  that  the  retina  and  disc  have  a  faint 

1  Stephen  Mackenzie,  Tram.  Ophth.  Soc.  U.K.,  1882,  ii.,  40. 

2  de  Schweinitz,  Trans.  Amer.  Ophth.  Soc.,  vii.,  655. 


70  LEUKEMIA 

opalescent  haze  over  them  as  if  they  had  been  smeared  over  with 
milk  and  water.  This  is  probably  an  expression  of  the  oedema 
described  by  de  Schweinitz  and  by  Sargent.1 

Ransome  and  Mules  2  state  that  the  tendency  to  retinal 
haemorrhages  commences  when  the  corpuscles  have  dropped  to 
32  per  cent,  or  thereabout. 

LEUKAEMIA. 

The  retina,  choroid  and  orbit  are  all  at  different  times  the 
seat  of  lesions  in  this  disease. 

Changes  in  the  f undus  as  seen  by  the  ophthalmoscope  are  so 
frequent  as  to  be  usual,  and  few,  if  any,  patients  die  of  leukaemia, 
without  at  some  time  showing  ophthalmoscopic  evidence  of  it, 
and  this  is  true  both  of  the  lymphocytic  and  of  the  myelogenic 
varieties,  whether  they  run  an  acute  or  a  chronic  course. 

Incidence. — Of  twenty-five  patients  suffering  from  leukaemia 
whom  I  examined  from  time  to  time,  twenty  were  of  the 
myelogenic  variety  and  five  of  the  lymphocytic. 

Thirteen  were  females  and  twelve  males.  The  average  age 
of  the  females  was  37-5  and  of  the  males  thirty-two  ;  the 
youngest  patient  was  a  boy  of  eight  suffering  from  the  acute 
lymphocytic  disease. 

In  the  myelogenic  disease  the  average  number  of  red  cor- 
puscles wa.s  2,986,700,  and  of  white  484,700  ;  in  the  lymphocytic 
variety  the  numbers  were  2,250,000  and  35,200. 

Amongst  these  twenty-five  patients  there  were  three  only- 
one  lymphocytic  and  two  myelogenic — in  whom  the  fundus 
was  entirely  free  of  any  change,  though  in  some  of  them  it  was 
slight. 

The  Ophthalmoscopic  Changes. — The  earliest  and  least  con- 
spicuous of  the  changes  is  an  engorgement  of  the  retinal  veins, 
no  other  abnormality  being  present. 

In  two  only  of  the  twenty -five  cases  could  it  be  said  that  the 
veins  were  certainly  engorged  to  a  degree  which  was  patholo- 
gical, and  that  no  other  change  was  present. 

1  Archiv.  ofOphth.,  1892,  xxi.,  45. 

2  Brit.  Med.  Jour.,  1887,  i.,  1112. 


THREE  GRADES  OF  CHANGE  71 

Of  the  two  sets  of  vessels  the  veins  are  engorged  more  often 
and  to  a  greater  degree  than  the  arteries  ;  thus,  of  the  cases  in 
which  the  veins  were  engorged,  the  arteries  also  showed 
engorgement  in  44  per  cent.  ;  engorgement  of  arteries  alone 
does  not  occur. 

What  may  be  called  the  second  stage  of  the  changes  is  by  a 
long  way  the  most  common.  It  was  present  in  four  out  of  five 
lymphocytic  cases,  and  in  twelve  out  of  twenty  of  the  myelo- 
genic  ;  thus  it  occurred  in  73  per  cent,  of  those  cases  in  which 
ophthalmoscopic  changes  were  present. 

In  this  stage  either  the  veins  alone  or  the  veins  and  arteries 
are  distended,  both  sets  of  vessels  are  paler  than  normal,  and 
they  come  to  approximate  each  other  in  colour,  and  retinal 
haemorrhages  are  present.  The  vessels  look  as  if  they  contain 
diluted  blood,  and  this  appearance,  together  with  the  fact  that 
there  is  less  difference  than  normally  between  the  colour  of 
arteries  and  veins,  is  an  expression  of  the  anaemia,  and  may  be 
seen  in  any  case  in  which  marked  anaemia  is  present. 

The  veins  are  sometimes  greatly  engorged,  turgid,  and 
sausage-like  ;  they  are  cut  into  where  they  are  crossed  by  arteries, 
but  are  not  displaced  laterally  as  occurs  in  arterio-sclerosis. 

The  arteries  less  frequently  show  marked  engorgement,  but 
not  to  the  same  extent  as  may  occur  in  the  veins. 

The  haemorrhages  vary  much,  they  may  be  very  extensive, 
or  a  careful  exploration  of  the  fundus  may  be  necessary  to  find 
one.  Sometimes  they  tend  to  surround  the  vessels,  but 
frequently  there  is  no  particular  arrangement  of  them. 

They  are  small  or  medium  in  size  for  the  most  part,  and  may 
be  flame-shaped  ;  or,  if  in  the  deeper  retinal  layers,  roughly 
circular  or  rosette -shaped  ;  they  may  burst  the  external  limiting 
membrane  and  become  subretinal. 

They  not  infrequently  tend  to  be  diamond-shaped  with  a  pale 
centre,  reminding  one  a  little  of  an  endothelial  cell  with  its 
nucleus,  and  whilst  this  particular  type  of  haemorrhage  is 
more  often  seen  in  leukaemia  than  in  other  conditions,  it  is  not 
limited  to  this  disease. 

The  appearance  seems  to  be  due  to  the  aggregation  of  leuco- 
cytes in  the  central  parts  of  the  haemorrhage. 


72 


LEUKEMIA 


The  occurrence  of  spontaneous  bleeding  from  the  iris  and 
cilary  body  has  been  described.1 

The  presence  of  retinal  haemorrhages  does  not  imply  a  more 
than  usually  grave  prognosis,  for  it  is  not  rare  to  find  a  complete 
clearing  of  the  fundus  during  a  temporary  improvement  in  the 
patient's  condition. 

What  we  may  term  the  third  stage  in  the  retinal  changes, 
consists  of  a  general  leukocytic  infiltration  of  the  retina  and 


Fro.  26. — A  section  through  the  choroid  and  a  part  of  the  sclerotic  from 
a  case  of  leukaemia.  There  is  profuse  infiltration  of  the  choroid, 
causing  very  great  thickening  of  it. 

choroid  in  addition  to  the  changes  previously  described  ;  as  a 
consequence  of  this  infiltration  the  fundus  gives  a  pale  or 
greemsh  reflex. 

The  term  "  leukaemic  retinitis  "  should  be  restricted  to  this 
condition.  It  is  not  of  common  occurrence,  for  of  the  twenty- 
five  cases  referred  to  it  was  present  in  four  only. 

The  veins  may  show  broacTwmTie  lines  along  them  from  peri- 
vascular  infiltration  ;  the  lines  may  be  as  broad  as  the  vein  itself, 
and  are  widest  along  the  convexities  of  the  curves  of  the  veins. 

1  Sorger,  Miinch.  med.  Wochensch.,  1898,  xlv.,  1120. 


INFILTRATIONS  OF  THE  ORBIT  73 

The  disc  edges  are  blurred  from  oedema.  Hudson  x  describes 
disorganisation  and  degeneration  of  the  nerve  fibre  layer,  and 
the  formation  of  cytoid  bodies  and  the  development  of  varicose 
nerve  fibres,  and  these,  together  with  masses  of  leukocytes, 
give  rise  to  the  white  areas  seen  with  the  ophthalmoscope  :  see 
also  Edmunds.2 

If  the  changes  are  severe,  narrowing  of  the  visual  fields  and 
nyctalopia  may  occur.  Deafness  is  sometimes  present. 

In  a  few  cases  leuksemic  tumours  develop  under  the  con- 
junctiva causing  a  brawny  mass  to  protrude  between  the  lids,3 
or  similar  growths  occur  in  the  lachrymal  glands  or  orbits, 
causing  proptosis  and  limitation  of  movements. 

Leuksemic  infiltrations  of  the  orbit  are  reported  by  Leber,4 
Kerschbaumer,5  Werner  6  and  Dunn.7  Lagrange  8  has  collected 
twenty-five  cases  of  lymphoma  of  the  orbit,  and  he  points  out 
that  the  symmetrical  nature  of  the  tumours  is  a  fundamental 
characteristic.  Cases  of  subconjunctival  infiltration  are  reported 
by  Meller  3  and  Hudson.9 

Two  views  are  put  forward  to  account  for  the  origin  of  the 
leukocytic  infiltration  of  the  retina,  choroid  and  orbit. 

Stock  10  and  Kerschbaumer  1X  look  upon  passive  infiltration 
by  diapedesis  and  local  proliferation  as  the  source,  whereas 
Tiirk  and  Meller  attribute  them  to  stimulation  and  overgrowth 
of  pre-existing  lymphoid  elements. 


CHLOROMA. 

This  is  a  rare  aggressively  growing  tumour,  having  a  bright 
green  colour,  and  showing  an  especial  predilection  for  the  bones 

1  Roy.  Land.  Ophth.  Hasp.  (Moorfields)  Reports,  1912,  xviii.,  153. 

2  Trans.  Ophth.  8oc..  U.K.,  1890,  x.,  157. 

3  Meller,  Archiv.  f.  Ophth.,  1906,  Ixii.,  130. 
*  Klin.  Monate.f.  Augen.,  1869,  vii.,  312. 

5  Archiv.  f.  Ophth.,  1895,  xli.,  99. 

6  Trans.  Ophth.  Soc.  U.K.,  1905,  xxv.,  181. 

7  Trans.  Coll.  Phi/sic.,  Philadelphia,  1893,  xv.,  103. 

8  Traites  des  Tumeurs  de  I'Oeil,  1904,  ii.,  466. 

9  Loc.  cit. 

10  Klin.  Monats.  f.  Augen.,  1906,  xliv.,  328. 

11  Loc.  cit. 


74  CHLOROMA 

of  the  skull  and  dura  mater  ;  dissemination  in  the  viscera 
occurs,  but  the  nervous  system  is  rarely  involved. 

There  is  a  difference  of  opinion  in  regard  to  the  exact  patho- 
logy of  the  condition.  Some  authorities  regard  the  disease  as 
essentially  a  leukaemia  with  secondary  malignant  tumours  ; 
others  regard  it  as  a  malignant  disease  of  bone  marrow  with 
secondary  changes  in  the  blood,  these  changes  being  in  the 
direction  of  a  lymphaemia  or  more  rarely  of  a  myelaemia. 

There  are  greenish  thickenings  of  the  skull  bones,  especially 
of  the  frontal  bones,  so  that  proptosisteusually  a  marked 
feature — thirty-one  out  of  forty-two  cases  (Pfeiffer)  ;  the  dura 
mater  becomes  infiltrated  in  a  patchy  manner  by  the  greenish 
tissue,  which  likewise  protrudes  into  the  lumen  of  the  venous 
sinuses  in  which  greenish  clots  may  be  found. 

This  green  colour  tends  to  bleach  in  museum  specimens,  but 
may  be  preserved  for  years  if  they  are  protected  from  the  light.1 

Affections  of  hearing  and  discharge  from  the  ears  are  common. 

Hudson  and  Coats  2  report  a  case  very  fully,  and  Dudgeon, 
who  examined  it  pathologically,  says  the  tissues  are  infiltrated 
with  myelocyte's  as  seen  in  the  tissues  and  blood  in  leukaemia . 

Turk  reported  a  case  in  which  green  deposits  were  scattered 
in  the  periosteum  of  the  lumbar^vertebrae,  and  in  which^47  per 
cent,  of  the  white  cells  in  the  blood  were  myelocytes^ 

The  close  association  of  chloroma  with  leukaemia  also  receives 
the  support  of  Fabian,3  Lehndorff,  Frith  and  Ledingham.4 

The  question  is  well  reviewed  by  Hall,  Hebb,  and  Bernstein.5 

Hudson  summarises  thus  :  "in  some  cases,  at  any  rate  .  .  . 
cells  which  are  normally  almost  confined  to  the  bone  marrow, 
and  which  have  an  important  relation  to  the  white  cells  of  the 
blood,  can  be  shown  to  form  the  most  prominent  constituents 
of  the  infiltration,  a  fact  which  suggests  that  such  cases  at 
any  rate,  must  be  regarded  as  examples  of  a  disease  arising 
primarily  in  the  myelogenous  component  of  the  haematopoietic 
system." 

1  St.  Bartholomew's  Hospital  Museum  No.  437a. 

2  Roy.  Lond.  Ophth.  Hosp.  Reports,  1912,  xviii.,  181. 

3  Beit.  z.  path.  Anat.  u.  z.  allg.  Path.,  1908,  xliii.,  172. 
*  Pro.  Roy.  Soc.  Med.,  Path.  Sect.,  1910,  iv.,  60. 

6  Pro.  Soy.  Soc.  Med.,  Med.  Sect.,  1909,  ii.,  157. 


CHLOROSIS  75 

Gallemaerts  x  reports  a  case  in  which  dissemination  took 
place  into  the  retina  and  choroid  of  one  eye,  amongst  many 
other  tissues. 

CHLOROSIS. 

A  considerable  number  of  cases  of  pathological  change  in  the 
eye  fundus  have  been  reported  in  this  fast  disappearing  disease, 
but  whilst  chlorotic  subjects  are,  of  course,  not  immune  from 
other  conditions  which  cause  papilloedema,  it  may  be  urged 
that  particular  care  is  needed  before  papilloedema  occurring  in 
a  chlorotic  subject  is  attributed  to  the  blood  condition.  Never- 
theless, in  a  number  of  cases  the  changes  are  reported  to  have 
disappeared,  and  the  vision  to  have  been  restored  under  treat- 
ment by  iron,  and  the  subsequent  progress  of  the  patient  has 
confirmed  the  causal  relationship  of  the  two  conditions. 

On  the  other  hand  a  number  of  the  reported  cases  are  not 
convincing,  either  as  to  the  presence  of  the  fundus  changes,  or 
as  to  the  exclusion  of  causes  in  addition  to  the  chlorosis,  and 
the  occurrence  of  pseudo-papilloedema  requires  especially  to  be 
had  in  mind. 

The  change  which  is  most  often  described  is  a  simple  papill- 
oedema, but  in  some  cases  a  star  figure  at  the  macula  and 
haemorrhages  have  been  described  ;  as  has  been  pointed  out, 
retinal  haemorrhages  may  be  seen  in  any  severe  anaimia,  and 
have  no  special  significance.  By  Pagenstecher  the  occurrence 
of  the  disc  change  is  attributed  to  a  supposed"  increase  of  the 
intracranial  pressure. 

The  combination  of  disc  changes  with  some  of  the  concomitant 
symptoms  of  chlorosis,  headache  and  perhaps  vomiting,  may 
closely  counterfeit  a  cerebral  tumour.2 

1  Klin.  Monats.  f.  Aug.,  1914,  Hi.,  878. 

2  The  following  are  references  to  a  few  of  the  reported  cases  and  literature  : 
Ballaban,  Archiv.  f.  Aug.,  xli.,  281  ;   Gushing  and  Bordley,  Amer.  Jour,  of  Med. 
Scien.,  1908  ;  Bannister,  Jour,  of  Nerv.  and  Men.  Dis.,  N.  York,  xxv.,  874 ;  Bitsch., 
Klin.   Monats.  f.   Aug.,   xvii.,    144 ;    Gowers,    Brit.    Med.  Jour.,  1881,   i.,  797  ; 
Kingborn,  Montreal  Med.   Jour.,   1900,  42 ;  Litten   and  Hirschberg,  Brit.  Med. 
Jour.,  1885,  i.,  328  ;    Westcott  and  Pusey,  Archives  of  Ophth.,  1902,  23  ;   Posey, 
Trans.  Amer.  Ophth.  Soc.,  xii.,  284. 


70  SECONDARY  ANEMIAS 

FUNDUS  CHANGES   IN   SECONDARY   ANAEMIAS. 

It  is  common  to  find  changes  in  the  eye  fundus  in  patients 
who  are  severely  anaemic  from  any  cause,  whether  from  loss  of 
blood  or  as  a  part  of  a  severe  emaciation  or  cachexia. 

Thus  I  examined  a  heterogeneous  group  of  forty -three  patients 
in  St.  Bartholomew's  Hospital,  ill  of  different  serious  diseases, 
all  of  whom  were  markedly  anaemic,  with  a  view  to  discovering 
the  frequency  of  these  changes. 

The  average  number  of  red  blood  cells  was  2,336,000,  and  the 
average  haemoglobin  percentage  35-3. 

Amongst  them,  gastric  or  duodenal  ulcer,  whether  innocent 
or  malignant,  formed  the  greatest  number,  but  other  diseases 
represented  were  haemophilia,  haemorrhoids,  enteric,  epistaxis, 
intestinal  haemorrhage,  etc. 

Some  of  the  changes  have  reference  only  to  the  local  expres- 
sion of  the  bloodlessness,  others  are  more  discrete  and  con- 
spicuous. 

Amongst  the  former  group  are  : — 

(1)  A  general  pallor  of  the  fundus,  as  of  the  cheeks. 

(2)  The  blood  within  the  retinal   vessels   may   be   clearly 

lacking  in  colour  as  if  it  had  been  diluted,  and  conse- 
quently there  is  less  difference  than  is  normal  between 
the  colour  of  the  arteries  and  the  veins. 

(3)  The  retinal  vessels  are  well  filled,  and  indeed,  in  spite  of 

the  general  bloodlessness,  it  is  not  rare  for  them  to 
appear  somewhat  distended. 

(4)  In  severe  cases  there  is  often  the  appearance  of  a  faint 

general  haze  over  the  whole  fundus  which  is  most 
noticeable  over  the  disc  ;  it  is  somewhat  as  if  the  surface 
had  been  covered  with  a  wash  of  milky  water. 

The  more  conspicuous  changes  consist  in  the  presence  of 
retinal  haemorrhages  and  white  patches. 

Amongst  the  forty -three  patients  referred  to,  ten  had  haemor- 
rhages alone,  two  had  white  patches  alone,  and  in  four  both 
haemorrhages  and  white  patches  were  present. 

The  haemorrhages  are  seldom  if  ever  profuse,  and  in  many 


WHITE  PATCHES  IN   THE  RETINA  77 

cases  two  or  three  only  are  present ;  they  are  usually  in  the 
nerve  fibre  layer  and  close  to  the  disc  ;  they  give  rise  to  no 
symptoms  and  disappear  leaving  no  residue. 

Their  incidence  is  not  in  direct  relationship  with  the  degree 
of  anaemia  : — I  have  examined  a  small  boy,  a  hsemophilic,  who 
bled  to  death  from  biting  his  tongue  when  cracking  a  nut,  but 
no  hsemorrhages  were  present — six  brothers  had  all  bled  to 
death  from  trivial  causes;  another  haemophilic  had  1,200,000 
red  corpuscles  and  18  per  cent,  of  haemoglobin,  but  his  retina 
was  free  of  hsemorrhages,  so  also  was  the  retina  of  a  girl  who  had 
only  520,000  red  blood  cells  and  18  per  cent,  of  haemoglobin. 

Of  special  interest  are  the  soft-edged  white  patches,  which 
individually,  are  quite  indistinguishable  as  seen  with  the 
ophthalmoscope,  from  the  "  cotton  wool  patches  "  of  renal 
retinitis. 

They  were  present  in  six  of  the  forty-three  cases,  in  two  by 
themselves,  and  in  four  in  association  with  haemorrhages. 

They  are  situated  in  the  nerve  fibre  layer,  for  they  sometimes 
partly  obscure  a  retinal  vessel. 

What  is  their  histological  counterpart  I  am  unable  to  say ; 
perhaps  they  represent  patches  of  gangliform  degeneration  in 
the  nerve  fibre  layer. 

When  these  patches  and  retinal  haemorrhages  occur  together, 
the  ophthalmoscopic  picture  cannot  be  distinguished  from  that 
which  is  occasionally  seen  in  early  cases  of  renal  retinitis. 

The  white"  areas,  like  haemorrhages,  disappear  and  leave  not 
the  slightest  trace  behind  ;  the  shortest  time  in  which  I  have 
observed  one  to  fade  away  is  fifteen  days. 


PURPURA. 

Haemorrhages  into  the  retinal  tissues  are  not  rare  in  this 
disease,  they  are  most  commonly  situated  in  the  nerve  fibre 
layer  and  not  far  from  the  disc. 

They  do  not  carry  any  particular  prognostic  significance,  and 
run  the  course  of  similar  haemorrhages  in  other  similar  condi- 
tions, e.g.,  the  secondary  anaemias,  etc. 


78  BLINDNESS  FROM  LOSS  OF  BLOOD 

RAYNAUD'S   DISEASE. 

A  few  observers  have  described  spasm  of  the  retinal  vessels 
in  this  disease. 

On  a  number  of  occasions  I  have  examined  specifically  for 
this  condition,  but  in  no  case  was  there  any  evidence  of  its 
presence  at  the  time  of  examination,  and  this  is  in  accord  with 
the  findings  of  Blaauw,1  who  made  a  careful  investigation  of 
this  point. 

Batten  2  reports  retinal  haemorrhages  in  a  woman  who  had 
suffered  from  the  disease  for  eleven  years,  and  in  whom  two 
toes  had  become  gangrenous. 

SERIOUS   LOSS    OF    SIGHT    RESULTING    FROM 
LOSS    OF  BLOOD. 

It  is  common  for  patients  who  are  actually  dying  from  loss 
of  blood  to  say  that  everything  is  black  before  them,  but  apart 
from  this,  large  haemorrhages  result  in  some  cases  of  those  who 
recover,  in  serious  or  complete  permanent  loss  of  sight  in  one 
or  both  eyes. 

v^_^^     «J^^  \s  ^^^ 

By  a  long  way  the  commonest  cause  is  bleeding  from  the 
intestinal  tract,  and  next  most  frequently  uterine  haemorrhage  ; 
it  is  quite  unusual  for  it  to  occur  as  the  result  of  traumatic 
haemorrhage. 

During  the  late  war,  in  spite  of  the  very  numerous  cases  of 
profuse  bleeding,  I  did  not  see  any  such  case,  nor  have  I  been 
able  to  hear  of  any,  and  Grout 3  states  on  the  authority 
of  De  Wecker,  Kneis  and  Wilbrand  and  Saenger,  that  in 
the  Franco -Prussian  War  no  case  was  seen  where  visual  dis- 
turbances could  be  attributed  to  surgical  haemorrhage  alone. 
Carlini,4  however,  reports  a  case  from  severance  of  the  radial 
artery  whilst  wood  chopping. 

Chevallereau 5    collected    twenty-one    cases    resulting    from 

1  Sammlung    zwangloser  Abhandlunqen  aus    dem   Gebiele  der   Auqenheilkunde, 
1913,  ix.,  5. 

2  Trans.  Ophth.  Soc.  U.K.,  1910,  xxx.,  238. 

3  Archiv.  of  Ophth.,  1914,  xliii.,  234. 

4  La  Clinica  Oculist.,  1906,  2353. 
6  Archiv.  d"0phth.,  1903,  417. 


BLINDNESS  FROM  LOSS  OF  BLOOD  79 

uterine  haemorrhage,  of  which  nine  were  confinements  at  term 
and  ten  were  cases  of  abortion. 

The  onset  of  blindness  may  be  immediate  or  it  may  be  de- 
layed, and  the  two  eyes  are  by  no  means  necessarily  equally  or 
simultaneously  affected. 

Thus,  Fries  l  analysed  ninety-six  cases,  and  found  that  in 
25  per  cent,  there  was  immediate  loss,  and  in  23  per  cent,  the 
onset  was  delayed  for  periods  up  to  eighteen  days  or  longer. 

Of  his  cases  there  was  complete  and  permanent  loss  of  sight 
in  both  eyes  in  65  per  cent.,  and  in  10  per  cent,  one  eye  alone 
was  affected;  of  Chevallereau's  cases  eight  were  chiefly 
unilateral. 

It  is  notable,  too,  that  when  both  eyes  are  affected  there  is 
often  an  interval  of  days  between  the  dates  of  their  involvement. 

Although  the  usual  history  is  of  a  large  suddenjqss  of  blood, 
sometimes  a  prolonged  drain,  e.g.,  from  haemorrhoids,  may 
produce  it.2 

Complete  recovery  of  vision  does  not  occur  :  a  man  who  had 
had  bleeding  piles  for  ten  years  suddenly  went  nearly  blind ; 
his  red  blood  cells  numbered  4,000,000  per  c.m.,  and  his  haemo- 
globin was  35  per  cent.  ;  five  weeks  after  operation  for  his  piles 
these  figures  were  6,200,000  and  105  respectively  ;  his  plethoric 
appearance  was  in  striking  contrast  to  his  previous  flabby, 
chlorotic  aspect ;  his  vision,  however,  did  not  improve.3 

Some  improvement,  nevertheless,  is  not  rare.  Sweet  4 
reports  such  a  case. 

On  ophthalmoscopic  examination  in  the  early  stage  there  is 
slight  oedema  of  the  disc,  the  vessels  are  pale,  but  of  full  size, 
and  in  a  few  cases  a  small  haemorrhage  or  two  have  been  present ; 
later  on  atropITy  becomes  established,  the  disc  becomes  paler, 
and  the  vessels  become  permanently  reduced  in  size. 

Ulrich  5  and  Hoffmann 6  examined  the  retina  immediately 
after  the  occurrence  of  a  large  haemorrhage. 

1  Quoted  Gowers'  "Med.  Ophth.,"  1904,  225. 

2  Foster  Moore,  St.  Bartholomew's  Hospital  Reports,  1910,  xlvi.,  181 ;    Trows. 
Ophth.  Soc.  U.K.,  1911,  xxxi.,  39. 

3  Foster  Moore,  ibid. 

*  Trans.  Amer.  Ophth.  Soc.,  1903,  x.,  183. 

5  v.  Graefe's  Archiv.  f.  Ophth.,  1887,  xxxiii.,  ii.,  1. 

6  Klin.  Monats.f.  Augenheilh.,  1883,  171. 


80  ERYTHRMMIA 

The  patient,  a  woman  of  twenty-five,  had  had  a  hsemate- 
mesis  from  a  gastric  ulcer ;  the  fundi  were  pale  but  were  other- 
wise normal. 

Two  days  later  a  fresh  bleeding  took  place  and  the  retinae 
were  examined  ten  minutes  later. 

The  discs  were  extremely  pale  and  sharply  outlined,  the 
arteries  were  narrow  and  difficult  to  follow  to  the  periphery,  the 
veins  were  somewhat  distended,  but  at  the  disc  edge  they 
suddenly  lost  their  dark  red  colour,  and  from  there  as  far  as 
could  be  seen  outwards  they  became  a  bright  red  as  if  the  veins 
were  continued  into  arteries. 

It  would  seem  clear  that  the  severe  local  anaemia  hereby 
implied  might  well  result  in  permanent  damage  to  the  retina. 

The  approximation  in  colour  between  the  arteries  and  veins 
is  common  in  severe  anaemia. 

ERYTHRffiMIA  (VAQUEZ'S  DISEASE). 

This  disease  was  first  defined  by  Vaquez  l  in  1892.  Its 
features  are  :— 

(1)  The  presence  of  erythraemia  for  which  no  cause — e.g., 
congenital  heart  disease,  chronic  bronchitis  and  emphysema — 
can  be  found.     The  number  of  red  cells  is   usually  between 
seven  and  twelve^millions,  the  cells  being  of  normal  appearance. 
The   haemoglobin   is   seldom    increased   in   proportion   to    the 
number  of  cells,  hence  the  colour  index  is  low.     There  is  a 
moderate  degree  of  leucocytosis,  from  10,000  to  30,000. 2 

(2)  A  florid  cyanotic" appearance. 

(3)  Enlargement  of  the  spleen  and  liver. 

The  disease  usually  occurs  in  adults  between  the  ages  of 
thirty  and  sixty,  and  is  rather  more  frequent  in  males. 

It  is  probably  due  to  a  primary  hyperplasia  of  the  erythro- 
blastic  tissue  of  the  bone  "marrow.3 

Defects  or  abnormalities  of  vision  are  often  complained  of, 
and  marked  ophthalmoscopic  changes  are  frequently  present. 
It  is,  however,  clearly  shown  by  a  number  of  observers,  that 

1  Bulletin  Med.,  1892,  vi.,  849. 

2  Parkinson,  John,  Lancet,  1912,  1425. 

3  Parkes  Weber,  Quart.  Jour,  of  Med.,  1908,  ii.,  85. 


ERYTHR&MIA 


81 


there  may  be  a  very  serious  disturbance  of  vision,  approaching 
to  complete  blindness,  with  almost  no  ophthalmoscopic  changes. 

Thus  Friedenwald  l  calls  particular  attention  to  the  visual 
disturbances  which  sometimes  are  accompanied  by  fundus 
lesions,  but  at  other  times  occur  independently,  or  in  association 
with  ophthalmoscopic  changes  which  are  quite  inadequate  to 
explain  them. 

Osier  2  reports  a  case  of  "  failing  vision,  the  condition  of  the 
fundus  being  negative  with  the  exception  of  tortuosity  of  the 


FIG.  27.- — A  section  through  the  retina,  choroid,  and  part  of  the  sclerotic 
to  show  the  great  engorgement  of  the  choroid  with  blood  in  a  case  of 
erythraemia  (Vaquez's  disease).  From  a  man,  a  patient  of  Dr.  J.  H. 
Drysdale,  who  had  had  symptoms  for  seven  months.  The  chief 
ophthalmoscopic  sign  was  a  great  engorgement  of  the  retinal  veins. 
At  one  time  his  blood  count  was  as  follows  :  Red  blood  cells, 
12,580,000  ;  haemoglobin,  148  per  cent. ;  colour  index,  0-74  ;  white 
blood  cells,  25,000.  A.  Retina.  B.  Choroid.  C.  Sclerotic. 

vessels,"  and  Lucas3  a  case  who  became  quite  blind  although 
nothing  was  found  in  the  optic  discs  beyond  slight  hyperaemia. 

I  have  seen  a  woman  who  was  all  but  blind  in  one  eye,  and 
had  greatly  reduced  vision  in  the  other,  with  disc  changes  which 
would  not  have  suggested  nearly  so  great  a  loss  of  sight. 

Of  little  importance  is  the  congestion^  of  the  conjunctival 
vessels  which  gives  the  eye  a  bloodshot^  appearance  and  so 
simulates  conjunctivitis. 

1  "  Contributions  to  Med.  and  Biolog.  Research,"  1919,  i.,  495. 

2  Amer.  Jour.  Med.  Sci.,  1903,  cxxvi.,  187. 

3  Archiv.  Int.  Med.,  1912,  x.,  597. 


82  ERYTHRMMIA 

The  abnormalities  of  sight  are  numerous,  varied,  and  are 
often  indefinite. 

Lucas  1  found  some  disturbance  of  vision  in  thirteen  out  of 
forty  cases,  and  Christian  2  describes  ten  cases,  in  eight  of  which 
there  were  indefinite  visual  disturbances. 

The  symptoms  complained  of  are  such  as  flashes  of  light, 
photophobia,  sparks,  blurring  or  general  mistiness  of  vision 
(John  Parkinson),  whirling  objects,  black  spots,  scotomata, 
hemianopia,  diplopia,  etc. 

Friedenwald  reports  a  case  in  which  visual  symptoms  pre- 
ceded all  other  signs  of  the  disease  by  several  years. 

Ophthalmoscopic  changes  are  frequently  present,  and  may  be 
extreme  in  degree  ;  I  have  notes  of  four  cases  in  which  they  were 
well  marked  ;  other  cases  are  reported  by  Jackson,3  Parker,4 
Jackson,5  Dupuy-Dutemps  and  Lutembacher,6  Hutchin- 
son  and  Miller,7  Osier,8  Englebach  and  Brown9  and  Watson.10 
In  three  of  my  own  cases  there  was  marked  or  extreme  engorge- 
ment of  the  retinal  veins  with  haemorrhages,  the  arteries  being 
of  normal  size,  and  in  one  of  them  the  condition  simulated 
thrombosis  of  the  central  retinal  vein  so  closely,  that  one  was 
led  to  suggest,  that  prominent  cerebral  symptoms  which  were 
present,  might  be  due  to  thrombosis  of  the  cranial  sinuses,  as 
indeed  proved  to  be  the  case  at  the  autopsy. 

In  one  of  the  cases  the  disc  changes  differed  little  from  the 
papilloedema  of  a  cerebral  tumour. 

CYANOSIS   RETINAE. 

In  some  cases  of  congenital  heart  disease  abnormal  retinal 
appearances  are  seen. 

Posey  u  collected  eighteen  cases. 

1  Arrhiv.  Int.  Med.,  1912,  x.,  597. 

2  Amer.  Jour,  of  Med.  Sci.,  1917,  cliv.,  547. 

3  "  Ophthalmology,".  1907,  iv.,  i. 

4  Trans.  Amer.  Ophth.  8oc.,  1909—11,  xii.,  505. 

5  Trans.  Amer.  Ophth.  Soc.,  1906—8,  xi.,  36.3. 

6  Annal.  d'Ocul.,  1912,  cxlviii.,  81. 

7  Lancet,  1906,  i.,  744. 

8  Amer.  Jour.  Med.  Sci.,  1903,  cxxvi.,  187. 

9  Jour.  Amer.  Med.  Assoc.,  1906,  xlvii.,  1265. 
10  Liverpool  Med.  Chir.  Jour.,  1906,  xxvi.,  33. 

11  Trans.  Amer.  Ophth.  Soc.,  1903—6,  x.,  634. 


CYANOSIS  RET 'IN 'M  83 

The  conjunctival  and  anterior  ciliary  veins  are  distended,  ' 
the  retinal  vessels,  both  arteries  and  veins,  are  darker  than 
normal,  the  arteries  being  about  the  colour  of  normal  veins, 
and  the  veins  are  of  a  much  darker  hue. 

At  times  engorgement  of  the  vessels  involves  both  sets  of 
vessels,  whereas  in  other  instances  the  size  of  the  arteries  is  not 
altered  ;  retinal  haemorrhages  are  not  infrequent. 

Nagel 1  states  that  if  both  arteries  and  veins  are  broader  and 
darker  than  normal,  the  presence  of  a  communication  between 
the  two  sides  of  the  heart  is  assured,  whereas  if  the  darker 
coloration  and  tortuosity  are  limited  to  the  veins,  some  other 
lesion  exists  which  does  not  allow  of  the  mixing  of  venous  and 
arterial  blood. 

As  compared  with  the  retinal  changes  in  Vaquez's  disease, 
to  which  there  is  a  considerable  similarity,  retinal  haemorrhages 
may  be  very  profuse  in  the  latter  and  are  never  profuse  in 
cyanosis  retinae. 

The  arteries  are  but  little  changed  either  in  size  or  colour  in 
Vaquez's  disease,  and  the  veins  do  not  attain  so  deep  a  colour 
as  in  cyanosis. 


RETINAL  HAEMORRHAGES  IN  NEW-BORN  INFANTS. 

Retinal  haemorrhages  are  of  common  occurrence  in  new  born 
infants,  and  are  presumably  developed  during  the  process  of 
birth. 

Coborn  2  examined  histologically  the  eyes  of  thirty-seven 
infants,  the  oldest  of  whom  had  lived  twenty-two  days ; 
seventeen  of  them  had  haemorrhages  in  the  retina,  especially  in 
its  inner  layers  ;  they  have  also  been  examined  histologically 
by  v.  Hippel.3 

De  Vries  4  examined  112  infants  ophthalmoscopically,  and 
found  haemorrhages  in  11  per  cent.,  and  Konigstein  5  found 

1  Quoted  by  Posey,  Trans.  Amer.  Ophth.  Soc.,  1906,  x.,  636. 

2  Archive.?  of  Ophth,  xxxiii.,  3,  256. 

3  v.  (iraefe's  Archiv.,f.  Ophth.,  1898,  xlv.,  313. 
1  Ned.  Tydschr.  d.  Oeneesk,  1901,  i.,  6. 

5  Mediz.  Jahrbiicher  Wien,  1881,  47 — 70. 

6—2 


84 


RETINAL    VENOUS  THROMBOSIS 


twenty-nine  cases  of  retinal  haemorrhages  amongst  281  infants, 
i.e.,  about  10  per  cent. 

Naumhoff  found  the  percentage  as  high   as  25.     (See  also 
p.  188.) 

THROMBOSIS   OF   RETINAL   VEINS. 

Thrombosis  may  take  place  in  the  central  retinal  vein  itself, 
and  then  usually  occurs,  as  shown  by  Coats,1  within  or  at  no 


FIG.  28. — Complete  canalisation  of  the  central  retinal  vein  following  its 
thrombosis.     (Coats.) 

great  distance  from  the  lamina  cribrosa,  or  it  may  occur  in  one 
of  the  tributaries  of  the  vein  as  it  courses  in  the  retina,  and  in 
this  case  its  site  may  be  determined  by  the  presence  of  a 
sclerosed  artery  crossing  it. 

Its  occurrence  is  signalised  by  a  rather  rapid  loss  of  sight,  but 
this  is  by  no  means  so  sudden  nor  is  it  nearly  so  complete  as  in 
sudden  obstruction  to  the  arterial  flow. 

For  a  full  and  excellent  account  of  the  pathology  of  the  con- 
dition, the  articles  by  Coats  should  be  consulted. 

1  v.  Graefe's  Archiv.  /.  Ophth.,  1913,  Ixxxvi.,  341  ;  Roy.  Land.  Ophth.  Hasp. 
(Moorfields)  Reports,  1906,  xvi.,  65. 


CAUSATION  85 

Frequency. — Within  twelve  months  I  was  able  to  collect 
sixty-one  cases  of  this  condition  at  the  Moorfields  Eye  Hospital. 

These  were  under  the  care  of  the  twelve  surgeons  there,  and 
many  of  them  had  been  attending  at  intervals  for  a  number  of 
years ;  this  number,  therefore,  does  not  represent  one  year's 
supply,  but  shows  that  during  one  year  more  than  sixty-one 
individuals  who  were  the  subject  of  this  complaint  attended  at 
the  institution. 

These  sixty-one  individuals  I  examined  at  regular  intervals 
as  to  their  blood  pressure,  the  urine,  visual  fields,  visual  acuity, 
and  intra-ocular  pressure,  and  in  more  than  half  of  them  I 
traced  by  means  of  pencil  drawings  some  of  the  minute  changes 
which  occurred  in  the  veins  themselves,  in  the  haemorrhages, 
the  exudates  and  the  discs. 

Age  and  Sex. — In  most  cases  the  patients  are  past  middle 
age,  the  average  for  fifty-nine  of  them  being — males  sixty, 
females  sixty -two — and  six  only  were  younger  than  fifty  years. 
The  average  age  of  Coats'  patients  was  60-8  years,  the  oldest 
being  eighty-five,  and  the  youngest  thirty.  The  sexes  are 
equally  liable,  for  thirty-one  were  males  and  thirty  females  ;  of 
Coats'  cases,  however,  twenty-five  were  males  and  eleven  females. 

Causation. — In  a  great  majority  cf  the  cases  the  immediate 
cause  of  the  thrombosis  is  angio-sclerosis,  and  as  has  been 
remarked,  the  immediate""elfc!ting  cause  in  some  cases  of  tribu- 
tary thrombosis  appears  to  be  the  pressure  of  a  thickened  artery 
acting  upon  a  vein  whose  walls  are  already  diseased. 

One  need  only  remember  the  changes  which  are  readily 
visible  in  the  veins  at  the  arterio- venous  crossings  in  many  cases 
of  arterio-sclerosis  (Fig.  19),  to  feel  no  surprise  that  thrombosis 
of  the  vein  should  here  occur.  ^^ 

The  evidence  of  arterio-sclerosis  is  generally  conspicuous  to 
ophthalmoscopic  examination. 

In  addition,  the  systolic  blood  pressure  in  forty  of  the  sixty- 
one  patients  in  whom  it  was  taken  at  intervals,  was  on  an 
average  187  mm.,  in  eleven  only  was  it  less  than  160. 

Urine. — In  fifteen  out  of  fifty  patients,  albumen,  usually  in 
small  amount,  was  found  in  the  urine,  and  I  suspect  that  had 
more  frequent  examinations  been  made,  the  proportion  would 


86  RETINAL    VENOUS  THROMBOSIS 

have  been  increased,  for  in  a  number  of  them  a  single  examina- 
tion only  was  made  ;  in  many,  however,  repeated  investigations 
at  intervals  gave  a  negative  result. 

In  one  only  was  sugar  present. 

Ophthalmoscopic  Appearances. — The  veins  in  this  condition 
are  greatly  engorged,  turgid  and  tortuous  ;  they  are  dark  in 
colour,  and  profuse  haemorrhages  into  the  retina  are  present  in 
the  whole  of  the  territory  drained  by  them. 

Thus,  if  a  tributary  only  is  thrombosed,  the  territory  of  the 
retina  drained  by  it  is  profusely  spattered  with  haemorrhages, 
whilst  the  rest  of  the  retina  is  clear,  the  whole  retina  is  covered 
with  haemorrhages  when  the  central  vein  itself  is  involved. 

The  haemorrhages  are  for  the  most  part  in  the  nerve  fibre 
layer,  are  largest  in  the  central  regions,  and  are  not  infrequently 
fringed  by  white  exudate. 

In  the  course  of  time  they  completely  disappear,  and  may 
leave  no  sort  of  trace  behind  of  their  former  existence,  though 
pigmentary  changes  of  a  fine  texture  are  not  rare,  especially  at 
the  macula  region. 

The  fundus  appearances  in  some  cases  of  Vaquez's  disease, 
simulate  very  closely  the  ophthalmoscopic  appearances  of 
thrombosis  of  the  central  retinal  vein. 

Of  the  present  sixty-one  patients,  thrombosis  of  a  tributary 
occurred  in  twenty-eight,  and  in  thirty-three  the  main  vein  was 
blocked. 

As  is  to  be  expected,  the  progress  of  the  changes  are  more 
severe,  and  the  complications  more  marked,  in  cases  where  the ' 
main  vein  is  involved. 

The  Visual  Fields. — The  fields  of  vision  show  less  change  than 
might  well  be  expected. 

In  the  case  of  thrombosis  of  the  central  vein  there  is  con- 
centric contraction  of  the  field,  though  not  to  a  great  extent, 
sometimes  there  are,  in  addition,  irregular  defects  for  which 
there  is  no  obvious  explanation,  but  most  important  is  the 
almost  constant  presence  in  these  cases  of  a  central  or  a  para- 
central  scotoma,  which  is  responsible  for  the  great  fall  in  visual 
acuity.  Sometimes  this  scotoma  is  partial  only,  and  requires 
investigation  with  low  illumination  for  its  discovery  (Fig.  31). 


PROGRESS 


87 


When  a  branch  of  one  of  the  retinal  arteries  is  suddenly 
obstructed,  there  is  immediately  developed  a  corresponding 
sector  defect  in  the  field  of  vision  ;  when,  however,  a  tributary 
of  the  vein  is  blocked,  no  such  sector  is  produced,  though  some 
concentric  constriction  may  occur,  and  besides,  unless  the 
blocked  vein  is  directly  concerned  with  the  macula  area,  no 
central  scotoma  occurs  and  the  visual  acuity  is  not  spoiled. 

Progress. — As  already  stated,  the  central  visual  acuity 
where  the  main  vein,  is  concerned, 
is  usually  much  reduced,  owing  to 
the  presence  of  a  central  scotoma, 
and  whilst  in  some  cases  the  general 
condition  of  the  retina  improves, 
and  in  a  few  cases  good  sight  is 
restored,  as  a  rule  no  improvement 
of  vision  occurs,  but  on  the  con- 
trary, there  is  a  further  slow  de- 
terioration till  no  useful  vision 
remains. 

In  tributary  cases  the  loss  of 
vision  is  much  less  from  the  first, 
and  many  cases  have  and  retain 
good  or  normal  central  acuity. 

Many  cases  of  central  vein  throm- 
bosis develop  acute  glaucoma  in  the 
course  of  a  few  weeks  and  so  sight 
is  lost,  but  otherwise  in  many  a  slow 
degeneration  from  chronic  glaucoma  occurs,  the  disc  becoming 
deeply  excavated  ;  this  does  not  happen  in  cases  where  a 
tributary  only  is  thrombosed) 

The  average  interval  between  the  thrombosis  and  the  onset 
of  glaucoma  was  100  days,1  or  9-07  weeks  (Coats),  eighteen 
out  of  twenty  occurred  within  fifteen  weeks,  the  longest 
interval  being  forty  weeks  (Coats). 

In  many  recovered  cases  bunches  of  tortuoHs  veins  are 
developed  on  the  discs,  the  result  of  the  development  of  col- 
lateral channels  (Fig.  29)  between  the  choroidal  and  retinal 

1  Inouye,  Royal  Lond,  Ophth.  Hosp.  Reports,  1910,  xviii.,  33. 


FIG.  29. — To  show  the  formation 
of  new  collateral  veins  on  the 
disc  in  a  man  of  sixty-two,  in 
whom  thrombosis  of  the  central 
retinal  vein  had  occurred  thir- 
teen months  previously.  His 
systolic  blood  pressure  was 
180  mm. 


• 


88 


RETINAL    VENOUS   THROMBOSIS 


systems ;  in  other  cases  circulation  is  restored  by  canalisation 
of  the  clot  as  shown  by  Coats  (Fig.  28). 

In  tributary  cases  it  is  very  interesting  to  watch  the  gradual 
formation  of  a  new  vessel,  always  exeedingly  tortuous,  to 
connect  the  distal  with  the  proximal  portion  of  the  vein,  the 
actual  thrombosed  part  being  transformed  into  a  fibrous  cord 
(Fig.  32),  but  in  addition,  extensive  development  of  new 
venous  channels,  or  perhaps  dilatation  of  existing  capillary 


AUC-  iO 


NOV.  29 


FIG.  30. — A  case  of  thrombosis  of  the  upper  temporal  vein  of  the  left  eye 
in  a  woman  aged  fifty-nine,  whose  systolic  blood  pressure  was 
155  mm.  Eight  and  a  half  years  later  she  was  in  good  health  ;  she 
described  the  left  eye  as  being  weaker  than  the  other,  a  blot  being 
over  the  inner  side.  The  first  drawing  was  made  five  months  after 
the  occurrence  of  symptoms  and  the  second  one  three  months  later. 
:  Notice  the  throwing  out  of  tortuous  venules,  apparently  in  an 
endeavour  to  establish  a  collateral  circulation.  See  also  Fig.  31. 

connections  occurs,  so  that  neighbouring  veins  become  con- 
nected by  fine,  very  tortuous  vessels,  the  useful  function  of 
which  is  not  always  clear. 

In  other  cases  these  vessels  appear  to  begin  to  develop,  and 
after  extending  for  considerable  distances  they  seem  to  end 
blindly,  having  apparently  failed  to  meet  with  a  reciprocal 
communicating  vessel  from  a  neighbouring  vein  (Fig.  30). 

The  prognosis  as  to  life  is  dependent  upon  the  general  con- 
dition of  the  patient  and  is  not  varied  by  the  accidental  occur- 
rence of  thrombosis  of  the  ocular  vein. 


BILATERAL   CHANGES 


89 


I  have  had  patients  under  observation  and  in  good  health, 
one  for  twenty-one  years  and  one  for  fifteen  years  after  the 
occurrence  of  thrombosis,  and  many  others  I  have  watched 
in  reasonable  health  for  many  years. 

It  is  not  common  for  a  patient  to  develop  venous  thrombosis 
in  each  eye,  even  though  the  factors  conducive  to  its  occurrence 
are  almost  necessarily  bilateral.  I  have,  however,  seen  it  occur 


oo~ 


-=-00 


135 


FIG.  31.- — The  visual  field  of  the  eye  from  which  Fig.  30  was  drawn. 
The  continuous  line  represents  the  field  taken  with  a  10  mm.  white 
square  in  full  daylight ;  the  dotted  line  represents  the  field  taken 
with  a  5  mm.  white  square  in  a  semi-darkened  room.  Note  the 
paracentral  scotoma,  and  that  there  is  no  sector  defect  of  field  to 
correspond  with  the  thrombosed  vein. 

on  five  occasions,  and  short  of  it,  haemorrhages  in  the  retina 
of  the  other  eye  are  common. 

On  several  occasions  I  have  found,  on  examining  the  "  good 
eye,"  that  innumerable  minute  haemorrhages  were  present 
towards  the  periphery  in  the  region  of  the  small  venous  radicles, 
looking  likes  leaves  on  the  branches  of  a  tree,  the  branch  being 
represented  by  the  smaller  veins  and  their  tributaries. 


90 


CAVERNOUS  SINUS  THROMBOSIS 


It  seems  likely  in  such  a  case  that  partial  obstruction  exists 
as  in  cases  reported  by  Coats,1  and  that  perhaps  a  collateral 
circulation  is  established. 

I  have  seen  one  such  case  in  which  seven  years  later,  only 
an  odd  haemorrhage  or  two  were  present,  and  the  vision  was 
6/6  ;  no  further  advance  of  the  disease  had  occurred. 

It  will  be  seen  that  a  very 
great  majority  of  the  cases 
under  consideration  occur  in 
folk  past  middle  age,  and  are 
directly  referable  to  disease 
of  the  vessels  ;  in  a  few  in- 
stances, however,  the  condi- 
tion occurs  in  youngsubjects 
without  any  evidence  of  vas- 
cular disease,  and  in  whom 
there  ^is  a  history  of  some 
recent  febrile  disorder. 

It  has  been  shown  by  Coats 

that  in  such    cases   the  veins 
show    evidence    of    an    acute 


FIG.  32.-To  show  the  formation  of  new 
collateral  venous  channels  in  the  retina 
consequent  upon  thrombosis  of  a  tribu- 


,   ,  ...  ,    .,     .  . 

tary  of  the  vein.     From  a  man  aged    phlebitis,    and    it    IS    at    times 
eighty-four    who   nineteen   years   pre-     difficult  to  avoid   the   COllclu- 

that     infection     of     the 


viously  had  attended  the  Moorfields 
Eye  Hospital  on  account  of  thrombosis 
of  the  vein.  A.  Thrombosed  vein  con- 
verted  into  a  fibrous  cord. 


son, 

•  11     • 

vem    wal1    1S 

cause  of  the  clotting.    It  is 

clear  that  such  an  occurrence  has  .^nb  prognostic  significance  as 
regards  the  future  health  of  the  individual.] 

THROMBOSIS   OF   THE   CAVERNOUS   SINUS. 

The  causes  of  thrombosis  of  the  cavernous  sinus  are  of  three 
kinds  :  — 

(1)  Infective. 

(2)  Primary  or  marasmic. 

(3)  Traumatic. 


Roy.  Land.  Ophth.  Hosp.  Report*.,  xvi.,  81. 


SIGNS  AND  SYMPTOMS  91 

The  changes  in  the  eyes  which  are  referable  to  its  develop- 
ment may  be  divided  into  two  groups  : — 

(1)  Those  due  to  obstruction  of  the  venous  circulation. 

(2)  Those  due  to  nerve  involvement. 

(1)  A  varying  degree  of  proptosis.  is  of  constant  occurrence, 
and  is  due  to  venous  obstruction  and  oedema  of  the  orbital 
cellular  tissues.  The  proptosis  commences  on  one  side,  but 
afterwards  spreads  to  the  opposite  side,  from  extension  of  the 
thrombosis  by  means  of  the  circular  sinus. 

This  is  a  point  of  importance  in  distinguishing  the  condition 
from  orbital  cellulitis,  which  it  may  simulate  closely,  and  with 
which  it  may  be  associated. 

Chemosis  and  oedema  of  the  lids  is  common.  Changes  in  the 
eye  fundus  are  by  no  means  constant ;  a  number  of  cases  are 
reported  in  which  oedema  of  the  retina,  swelling  of  the  disc, 
and  tortuous  veins  have  been  present ;  on  the  other  hand,  the 
fundi  are  frequently  free  of  any  marked  abnormality. 

It  was  shown  by  Wadsworth  and  Putman  that  no  certain 
change  was  observable  in  the  retinal  veins,  when  an  extreme 
degree  of  vascular  congestion  was  produced  by  tying  a  handker- 
chief round  the  neck. 

Coupland  l  suggests  that  when  retinal  changes  are  present 
they  imply  that  the  thrombotic  process  has  spread  to  the  facial 
or  ophthalmic  veins  .^ 

(2)  All  thB"  ocular  nerves  are  apt  to  be  involved  as  they 
traverse  the  cavernous  sinus,  producing  supra-orbital  neuralgia, 
ptosis,  and  ophthalmoplegia  interna  and  externa. 

This  is  especially  true  of  infective  cases,  and  occurs  also  in 
orbital  cellulitis.  It  is  a  point  which  may  be  of  considerable 
importance  in  distinguishing  one  or  other  of  these  conditions, 
from  the  proptosis  due  to  a  neoplasm  or  Graves'  disease. 

In  non-infective  cases  the  function  of  the  nerves  is  often 
spared. 

Frequency.— Coupland  collected  twenty-eight  cases,  in 
twenty-three  of  which  a  post-mortem  examination  was  made. 

1  Trans.  Ophth.  Soc,  U.K.,  1887,  vii.,  240. 


92  CAVERNOUS  SINUS  THROMBOSIS 

Fourteen  were  of  infective  origin,  and  seven  of  these  were  due 
to  otitis  media. 

Four  were  traumatic,  and  five  were  included  under  the  heads 
of  marasmus,  cachetic,  or  primary. 

Jackson  1  added  twenty-eight  cases  to  182  collected  by  D  wight 
and  Germain,  and  showed  that  of  these  210  cases,  20  per  cent, 
were  secondary  to  infection  in  the  region  of  the  face. 

1  "  Ophthalmology"  1909,  v.,  185. 


CHAPTER  III 

Disturbances  of  visual  orientation — Lesions  of  the  sympathetic  nerve — 
Hysteria — Epilepsy — Ophthalmoplegia  externa — Ophthalmoplegia 
interna  of  unknown  cause — Hydrocephalus — Encephalitis  lethar- 
gica — Polio -encephalitis — The  intra-ocular  lesions  in  tabes  and 
general  paralysis — Friedreich's  ataxia — Acute  myelitis  with  retro- 
bulbar  neuritis — Disseminate  sclerosis — Syringomyelia — My  as- 
thenia gravis — Myotonia  atrophica. 

DISTURBANCES   OF   VISUAL   ORIENTATION. 

LESIONS  of  certain  parts  of  the  brain  have,  as  a  prominent 
manifestation,  great  disturbance  of  visual  orientation,  and 
whilst  these  have  been  noted  previously,  it  is  only  as  a  result  of 
the  investigation  of  wounds  of  the  brain  in  the  late  war  chiefly 
by  Gordon  Holmes  1  and  Holmes  and  Horrax  2  that  the  locali- 
sation of  the  lesion  has  been  identified,  and  the  clinical  signs 
defined. 

Seven  of  such  cases  were  studied  minutely.  The  following 
were  the  chief  manifestations  of  the  defect. 

The  patient  wasHinable  to  touch  accurately  an  object  within 
his  sight  and  reach,  and  in  attempting  to  do  so  he  made  mistakes 
in  all  three  dimensions  of  space  :  he  would  collide  with  easily 
seen  objects  ;  if,  however,  he  was  allowed  to  touch  them  he 
would  easily  evade  them. 

t/The  absolute  and  relative  size  and  position  of  two  objects 
simultaneously  in  the  visual  field  were  not  accurately  judged. 
n  Such  men,  too,  show  a  difficulty  in  exploring  a  limited  surface 
with  their  eyes  ;  thus  they  are  unable  to  count  the  number  of 
coins  placed  on  a  table,  and  for  this  same  reason  reading  is 
difficult.  Nv 

In  a  few  cases  stereoscopic  vision  seems  to  be  destroyed.3 

There  is  a  difficulty  in  the  fixation  of  objects  seen  ;  this  is  a 
natural  result  of  the  fact  that  the  position  of  an  object  throwing 

1  Brit.  Jmir.  of  Ophth.,  1918,  ii.,  449  and  506. 

2  Archiv.  of  Neurol.  and  Psych.,  1919,  i.,  385. 

3  Riddoch,  Brain,  1917,  xl.,  45. 


94  SITE  OF  CEREBRAL  LESION 

an  image  on  the  retina  is  not  correctly  appreciated,  and  conse- 
quently the  eyeballs  cannot  be  so  rotated  as  to  bring  the 
exciting  image  to  the  macula. 

Site  of  the  Cerebral  Lesion. — In  two  cases,  Gordon  Holmes 
was  able  to  examine  the  brains  post-mortem ;  in  the  other 
cases  the  site  of  the  damage  was  estimated  by  craniometric 
measurements. 

He  believes,  that  in  order  to  produce  this  symptom  complex, 
the  lesion  must  involve  that  part  of  the  lateral  surface  of  the 
hemisphere  which  includes  the  angular  and  supra-marginal 
gyri,  and  extends  into  the  adjoining  occipital,  temporal,  and 
parietal  convolutions,  and  that  the  lesion  must  be  a  bilateral 
one. 

The  wounds  on  the  mesial  surface  of  the  hemisphere  probably 
all  lay  in  the  region  of  the  splenium.  In  several  cases  there 
were  defects  of  the  visual  fields  due  to  damage  to  the  optic 
radiations. 

For  several  reasons  it  seems  clear  that  the  symptoms  are  due 
to  the  damage  to  the  cortex  on  the  lateral  surface  of  the  hemi- 
sphere, or  to  the  subjacent  fibre  system,  and  not  to  the  lesion  on 
the  mesial  surface,  for  a  few  cases  showing  these  clinical  mani- 
festations have  been  recorded,  which  were  due  to  vascular 
lesions  in  this  region  on  both  sides,  but  without  damage  to  the 
mesial  surfaces  ;  and  further,  certain  manifestations,  which  were 
almost  undoubtedly  due  to  defects  of  visual  orientation,  were 
observed  in  monkeys  after  the  removal  of  corresponding 
cortical  areas  on  both  sides,  by  Monk,  and  Ferrier  and  Schafer, 
without  interference  with  the  mesial  aspect  of  the  hemisphere. 

LESIONS   OF  THE   SYMPATHETIC   NERVE. 

^The  nerve  fibres  which  control  the  dilator  pupillse  are  believed 
by  some  to  commence  their  course  in  the  medulla,  and  by  others 
to  start  from  a  cilio-spinal  centre  in  the  cervical  cord.  Karplus 
and  Kreidl,1  however,  as  a  result  of  experimental  work,  seem 
to  have  established  the  existence  of  an  important  sympathetic 
centre  in  the  hypothalamic  region. 

1  Arch./,  d.  ges.  Physiol,  1909,  cxxix.,  138  ;  cxxxv.,  401,  and  cxliii.,  109. 


LESIONS  OF   THE  SYMPATHETIC  NERVE    95 

From  this  centre  fibres  go  to  both  sympathetic  nerves,  but 
especially  to  the  contra -lateral  one,  and  Spiller  l  believes  on 
clinical  grounds  that  in  man  the  decussation  takes  place  above 
the  pons. 

The  fibres  leave  the  spinal  cord  by  the  anterior  roots  of 
probably  the  last  cervical  and  first  two  dorsal  nerves,  although 
'  the  exact  roots  are  still  in  dispute  2  ;  they  reach  the  corre- 
sponding sympathetic  ganglia  by  means  of  the  white  rami 
communicantes,  and  continue  in  the  sympathetic  chain  as 
preganglionic  fibres,  to  the  superior  cervical  sympathetic 
ganglion. 

In  this  ganglion  synapses  are  formed  with  ganglion  cells  from 
which  the  post-ganglionic  fibres  arise. 

These  fibres  are  continued  into  the  skull  with  the  internal 
carotid  artery,  and  joining  the  medial  branches  of  the  internal 
carotid  nerve,  connect  up  with  the  cavernous  plexus,  from 
whence  they  join  the  ophthalmic  division  of  the  trigeminus, 
and  by  the  naso-ciliary  and  long  ciliary  nerves  reach  the 
pupil. 

/  It  is  presumed  that  a  lesion  of  the  fibres  may  occur  in  any 
part  of  their  course,  and  may  be  of  an  irritative  or  paralytic 
'nature  ;  the  latter  is  much  the  more  frequent,  and  it  is  probable 
that  the  irritative  lesions  ultimately  pass  into  the  paralytic 
stage. 

Cases  have  been  reported  in  which  symptoms  of  irritation  of 
the  nerve  have  occurred  with  paralysis'oi  the  limbs.3 

Condition  of  the  Pupil. — With  paralysis  of  the  nerve  the  pupil 
is  smaller  than  normal ;  it  is  still  further  constricted  by  light 
stimulus  and  by  eserine,  and  is  dilated  by  atropine. 

Instillation  of  adrenalin,  ordinarily  inactive  in  causing 
dilatation  of  the  pupil,  becomes  exceedingly  active  when  the 
sympathetic  is  cut,  or  the  superior  ganglion  removed.4 

The  pupil  fails  to  dilate  with  cocaine,  consequently  the 
instillation  of  this  drug  in  a  5  per  cent,  solution  to  each  eye, 

1  Amer.  Jour.  of.  Med.  Scien.,  1920,  clix.,  325. 

2  Buzzard,  Brain,  1902,  xxv.,  308;  Bramwell,  Rev.  Neurol.  and  Psychiat.,  1903, 
i.,  236. 

3  Lloyd,  "  Twentieth  Century  Practice  of  Med.,"  1897,  xi.,  457. 
*  de  Schweinitz,  Jour.  Amer.  Med.  A **..  1904,  xlii.,  286. 


96  SIGNS  AND  SYMPTOMS 

and  comparison  of  the  pupils  at  the  end  of  a  quarter  of  an  hour, 
becomes  a  convenient  test  for  sympathetic  paralysis. 

Parsons  and  Henderson  showed  that  in  animals,  this  effect 
was  immediate  and  persisted  indefinitely  (two  years). 

Mayou l  and  Bistis  2  have  each  reported  cases  of  sympathetic 
'paralysis,  in  which  the  iris  on  the  affected  side  was  blue  or  grey, 
the  opposite  one  being  brown,  there  being  no  other  cause  for 
this  abnormality. 

The  size  of  the  affected  pupil  is  about  what  it  would  normally 
be  in  a  good  light,  and  so  the  abnormality  is  apt  to  be  overlooked  ; 
the  disparity  in  size  of  the  two  pupils,  however,  at  once  becomes 
obvious  in  dull  illumination. 

The  symptoms  of  irritation  of  the  nerve  are  : — 


(1)  Mydriasis  due  to  spasm  of  the  dilator  pupillae. 

(2)  Widening  of  the  palpebral  fissure  due  to  contraction  of 

unstriped  muscle  tissue  in  the  upper  lid. 

(3)  Constriction  of  the  vessels  of  the  face  and  neck  and 

increase  of  perspiration. 

(4)  And  perhaps  exophthalmos,  which  is  said  to  be  due  to 

the  contraction  of  unstriped  muscle  tissue  in  the  orbit 
(Muller's  muscle). 

The  paralytic  symptoms  are  the  converse  of  the  above. 
There  is  a  tendency  for  some  of  these  manifestations  to  be 
recovered  from  in  part  as  time  proceeds,  but  it  seems  that  the 
pupillary  changes  are  permanent.  Thus  it  was  shown  by 
Jessop  3  that  removal  of  a  piece  of  the  sympathetic  nerve  was 
followed  immediately  by  miosis,  and  Parsons  and  Henderson  4 
showed  that  the  above  was  true,  whether  the  nerve  was  cut  or 
the  ganglion  was  removed  ;  they  showed  further,  by  keeping 
the  animal  alive,  that  the  condition  of  the  pupil  was  unchanged 
at  the  end  of  two  years. 

Langley  and  Anderson  5  demonstrated  that  the  sympathetic 
has  no  inhibitory  power  over  the  sphincter  pupillse. 

1  Trans.  Ophth.  Soc.,  1910,  xxx.,  196. 

2  Arch,  of  Ophth.,  1915,  xliv.,  433. 

3  Pro.  Roy.  Soc.,  1885,  xxxviii.,  432. 

4  Ophth.  Review,  xxvii.,  325. 

5  Jour.  Physiol.,  1892,  xiii.,  555. 


ETIOLOGY  97 

They  were  unable  to  prove  the  existence  of  an  actual  dilator 
muscle  of  the  iris  as  occurs  in  some  animals.  Langley  says  l  : 
"The  contractile  substance  probably  consists,  not  of  ordinary 
unstriped  muscle,  but  of  the  processes  of  the  cells  covering  the 
posterior  surface  of  the  iris." 

Heese  2  states  that  the  sympathetic  has  no  control  over 
accommodation. 

Causes. — There  is  no  known  specific  cause  for  paralysis  of 
the  sympathetic,  but  it  seems  it  may  be  affected  by  any  patho- 
logical process  in  any  part  of  its  course  ;  amongst  the  causes 
are  the  following  :— 

Syringomyelia,  or  injuries  to  the  spinal  cord  ;  thus  Hutchin- 
son  3  reports  a  case  of  partial  division  of  the  cord  by  a  stab 
wound,  with  symptoms  of  sympathetic  paralysis  on  the  injured 
side  in  addition  to  the  limb  palsies  ;  the  patient  survived  for 
many  years,  the  symptoms  persisting": 

Injuries  of  the  roots  of  the  brachial  plexus  ;  involvement  in 
the  thorax  by  growths,  glands  or  aneurysms  ;  cervical  ribs 4 ; 
aneurysms,  growths  or  glands  in  the  neck  ;  simple  goitres,5  etc. 

In  addition,  many  cases  occur  in  which  there  is  no  discover- 
able cause,  or  in  which  the  only  discoverable  possible  cause, 
e.g.,  a  small  thyroid  adenoma  or  slight  enlargement  of  cervical 
glands,  etc.,  seems  to  be  inadequate  ;  indeed  the  majority  of 
cases  come  under  this  head. 

Glaucoma. — -A  number  of  observers  have  found  histological 
changes  in  the  sympathetic  ganglia  in  glaucoma,  and  this  has 
led  to  resection  of  the  nerve  for  the  relief  of  this  disease. 

Changes  were  found  by  Fisch,6  Ziehe  and  Axenfeld,  Lodato,7 
and  others. 

It  has,  however,  to  be  borne  in  mind  that  pathological  changes 
are  usually  to  be  found  in  these  ganglia  in  old  people  whatever 
the  cause  of  death. 

The  point  has  been  re-examined  by  Ira  Van  Giesen  and 

1  Schafer's  "  Text-book  of  Physiol.,"  1900,  ii.,  617. 

2  Phliiger's  Archiv.  f.  Physiol.,  1892,  Hi.,  535. 

3  Roy.  Lond.  Ophih.  Hosp.  Reports,  v.,  139. 

4  Mayou,  "  Ophthalmoscope,'''  191(5,  xiv.,  313. 

&  Langdon,  Trans.  Amer.  Ophih.  Soc.,  1916,  xiv.,  661. 

6  "  Ninth  Internat.  Ophth.  Congress,  Utrecht,  1899,"  5-44. 

7  Arch,  di  Ottalm.,  viii.,  358. 


98  HYSTERIA 

Weeks,1  who  examined  seven  ganglia  from  patients  with 
glaucoma,  and  four  other  ganglia,  to  act  as  controls,  were  carried 
through  all  the  processes  parallel  with  the  former. 

They  state  :  "  We  find,  then,  that  as  far  as  our  methods  of 
examination  of  hardened  material  go,  the  ganglia  seem  to  be 
normal  with  the  exception  of  an  excess  of  pigment  in  the 
neurone  cell  bodies,  and  as  to  the  significance  of  this,  if  it  has 
any,  we  are  entirely  as  yet  in  the  dark. 

"  The  result  of  the  examinations  does  not  positively  preclude 
the  existence  of  a  lesion  in  the  ganglia." 

HYSTERIA. 

The  most  important  manifestations  of  hysteria  as  they  affect 
the  eye,  are  los^of  visual  acuity,  and  alterations-'in  the  visual 
fields  ;  they  are  often  included  under  the  general  term  "  hys- 
terical amblyopia." 

Cases  occur  in  either  sex,  several  cases  being  recorded  in 
young  boys,  thus  Harlan  2  and  Galezowski  have  each  seen  it  in 
a  boy  of  eleven,  and  Moore  3  in  a  boy  of  fifteen. 

Gunn  4  divides  these  cases  into  two  classes  : — 

(1)  Idiopathic,  occurring  usually  in  females. 

(2)  Traumatic,   affecting  the  sexes  equally,   but   occurring 

usually  in  adults. 

Neuropathic  tendencies  are  generally  prominent  in  these 
patients. 

The  onset  of  symptoms  is  insidious  in  the  idiopathic  form, 
and  diagnosis  may  be  difficult. 

In  cases  following  trauma  there  may  be  a  period  of  incubation 
as  pointed  out  by  Charcot. 

The  loss  of  sight  is  alwayiTof  It  high  degree  and  is  most  often 
unilateral.  There  are  no  ophthalmoscopic  changes,  and  whilst 
both  Borel 5  and  Harlan  6  report  cases  in  which  it  is  stated  the 

1  Trans.  Amer.  Ophth.  Soc.,  1902,  ix.,  455. 

2  Trans.  Amer.  Ophth.  Soc.,  1888 — 90,  v.,  328. 

3  Trans.  Amer.  Ophth.  Soc.,  1888—90,  v.,  84. 

4  Brit.  Med.  Jour.,  1902,  ii.,  1425. 
Annal.  d'Ocul.,  1900,  cxxiii.,  5  and  241. 

6  Trans.  Amer.  Ophth.  Soc.,  1888—90,  v.,  330. 


FIELDS  OF    VISION  99 

pupil  reactions  were  abolished,  this  is  of  such  exceedingly  rare 
occurrence  as  not  to  vitiate  the  statement  that  the  pupil 
reactions  are  unchanged  in  hysteria. 

Much  stress  has  been  laid  upon  the  contraction  of  the  visual 
fields,  and  especially  upon  the  so-called  spiral  contraction  which 
was  believed  to  be  characteristic,  and  due  to  fatigue.  Hurst 
and  Symns l  have,  however,  cast  grave  doubt  upon  the  nature 
of  this  sign,  and  have  shown  how  it  is  produced ;  they  point 
out  that  it  is  a  product  of  the  perimeter,  and  that  if  some  less 
suggestive  means  be  employed  to  examine  the  fields,  no  constric- 
tion will  be  found.  They  show  further,  that  the  field  obtained 
is  a  narrowing  spiral  if  the  test  object  on  the  perimeter  is  carried 
from  without  in,  and  a  widening  spiral  if  the  object  is  carried 
from  within  out  ;  and  also  that  a  narrowing  spiral  may  be 
obtained  in  one  eye  and  a  widening  one  in  the  other  (Figs.  33  and 
34),  and  that  in  repeating  the  examination,  the  eye  which  before 
produced  the  narrowing  spiral  may  be  made  to  produce  a 
widening  one,  and  vice  versa.  They  state  "  we  have  never 
seen  patients  with  hysterical  symptoms  who  spontaneously 
complained  of  disabilities  resulting  from  a  narrow  field  of 
vision,  however  closely  they  were  cross-examined  on  the  sub- 
ject "  ;  further,  "  we  have  examined  numerous  soldiers  suffering 
from  various  war  neuroses,  some  of  whom  were  suffering  from 
gross  hysterical  symptoms,  and  we  have  never  found  any  restric- 
tion of  their  fields  of  vision  until  they  were  tested  with  the 
perimeter,  but  the  perimeter  invariably  resulted  in  the  suggestion 
of  a  narrowed  field,  however  carefully  it  was  used." 

The  fields  for  colour  are  less  constricted  than  for  white.2 

Many  cases  are  reported  where  the  amblyopia  has  persisted 
for  very  long  periods  without  change. 

Thus  Dupuy-Dutemps  3  reports  the  case  of  a  man  of  thirty  - 
eight  who  was  struck  in  the  face  with  a  piece  of  cloth,  and  who 
went  blind  in  the  left  eye  immediately  afterwards.  Six  and  a 
half  years  later  he  was  still  quite  blind  in  the  eye ;  he  did  not 
flinch  on  suddenly  approaching  an  object  to  the  eye  ;  patches 

1  Brit.  Jour,  of  Ophlh.,  1919,  18. 

2  Casey  Wood,  Amer.  Jour,  of  Med.  Sci.,  1899,  cxvii.,  42. 

3  Soci&e  francaise  d'opth.,  1902,  457. 

7—3 


100 


H YSTERIA 


of  cutaneous  anaesthesia  were  present.  He  quotes  a  second  case 
which  persisted  unchanged  for  a  year  and  a  half,  the  eyes  being 
quite  normal  in  every  way.  Harlan  reports  a  case  of  ten  years' 

duration. 

For  differential  diagnosis,  more  particularly  from  retrobulbar 
neuritis,  Gunn  lays  great  stress  on  the  dilatation  of  the  pupil 
which  immediately  succeeds  to  constriction  in  this  latter  disease  ; 


FIG.  33. — Spiral  field  of  vision  in  a  case  of  hysterical  mutism  and 
tremor.     Without-inward  spiral.     (Hurst  and  Symns.) 

he  insists  on  the  value  of  a  comparison  of  the  pupil  reaction  in  the 
sound  as  compared  with  the  diseased  eye,  and  on  the  consensual 
pupil  reactions.  Hemianopia  occasionally  occurs. 

Twice  I  have  seen  girls  who  produced  a  conjunctivitis  by 
putting  soap  into  their  eyes.  In  one  it  was  proved  by  pouring 
some  atropine  solution  on  the  soap  and  letting  it  dry  in  ;  next 
day  in  addition  to  the  conjunctivitis  the  pupil  was  dilated.  I 
have  seen  a  girl  cured  of  blindness,  by  suddenly  presenting  at 


101 

her  face  a  frog,  borrowed  from  the  physiological  laboratory  for 
the  purpose.  /• 

During  the  war  many  men  developed  severe  blepharospasm.^  xi 
A  few  went  completely  blind,  their  pupils  reacting  normally  ;  ^ 
some  of  them  were  quite  willing  to  see  and  rejoiced  on  the 
recovery  of  their  sight. 

Amongst   other   manifestations   may   be   mentioned  photo- 


FIG.  34. — Spiral  field  of  vision  in  a  case  of  hysterical  mutism  anl 
tremor.     Within-outward  spiral.     (Hurst  and  Symas.) 

phobia,  polyopia,  monocular  diplopia,  asthenopia,  and  other 
symptoms. 

EPILEPSY. 

Organic  changes  in  the  eyes  do  not  occur  with  true  epilepsy, 
the  presence  of  papillcedema  would  be  conclusive  that  epilepti- 
form  convulsions  were  dependent  upon  a  gross  cerebral  lesion. 

Subjective  symptoms  however,  such  as  aurse,  and  temporary 
alterations  in  the  pupils,  retinal  vessels,  and  external  muscles, 
occur  in  connection  with  true  epileptic  seizures. 


102  EPILEPSY 

Gowers  x  says  a  visual  warning  of  epilepsy  is  twice  as  common 
as  all  the  other  special  sense  aurse  together. 

There  may  be  sudden  loss,  or  dimness  of  sight,  flashes  or  balls 
of  coloured  light  may  be  seen,  or  objects  may  seem  to  grow 
smaller  or  larger,  to  recede  or  advance,  or  less  definable  or  highly 
complex  scenes  may  appear,  to  usher  in  the  seizure. 

Where  there  is  forced  rotation  of  the  head  during  an  attack, 
conjugate  deviation  of  the  eyes  towards  the  same  side  often 
occurs  ;  nystagmus  and  squint  are  also  sometimes  seen. 

The  Pupils. — During  the  fit  the  pupils  are  widely  dilated,  and 
except  in  very  mild  cases  are  inactive  to  light  stimulus. 

Spratling  2  says  he  does  not  recall  a  single  case  in  which  the 
pupils  were  not  dilated ;  it  is,  however,  stated  that  in  the  very 
beginning  for  a  short  time  they  are  small. 

Ophthalmoscopic  Alterations. — It  is  clear  that  there  are  no 
organic  f undus  changes  in  epilepsy,  but  it  seems  well  established 
that  during  the  stage  of  lividity  venous  engorgement  may  be 
evident  in  the  retina. 

Gowers  3  says  :  "I  have  examined  very  carefully  several 
thousand  epileptics,  and  have  found  in  most  cases  every 
character  of  the  fundus  to  be  such  as  is  presented  by  persons 
not  epileptic. 

"  In  a  case  of  convulsions  from  meningeal  haemorrhage,  and 
in  another  case  of  one-sided  fits,  I  have  been  able  to  keep  a 
retinal  artery  and  vein  under  direct  view  through  the  whole  of 
a  severe  fit,  from  before  its  commencement  till  after  its  close. 
In  neither  case  did  the  retinal  artery  present  the  slightest 
change  in  size.  During  the  stage  of  lividity  the  vein  became 
large  and  dark." 

Once  only  have  I  found  one  or  two  small  haemorrhages  in  the 
retina  after  a  fit,  and  presumably  caused  by  it ;  their  occurrence 
is  certainly  uncommon. 

At  different  times  by  different  observers,  errors  of  refraction 
have  been  held  responsible  for  most  extraordinarily  diverse 
morbid  manifestations,  from  the  delayed  healing  of  war  wounds 

"  Diseases  of  Nervous  System,"  1893,  ii.,  739. 
"  Epilepsy  and  its  Treatment,"  1904,  268. 
"  Medical  Ophthalmoscopy,"  1904,  191. 


OCULAR  DEFECTS  AS  A   CAUSE  103 

to  gastritis  ;  but  when  it  is  remembered  that  there  are  few 
individuals  in  whom  it  is  not  possible  to  find  at  least  some 
trivial  refractive  error  if  sufficient  assiduity  be  displayed,  it  will 
be  granted,  that  before  such  an  error  can  be  accused  of  acting 
as  an  excitatory  factor  in  the  production  of  epileptic  seizures 
in  a  predisposed  individual,  the  point  requires  especially 
critical  examination. 

The  best  answer  seems  to  be  supplied  by  a  Commission  of  the 
New  York  Neurological  Society  x  which  was  instituted  to  con- 
sider the  contention  of  Dr.  George  Stevens,  who  considered 
that  "  difficulties  with  accommodation  and  adjusting  the  eyes 
in  the  act  of  vision,  are  amongst  the  most  prolific  sources  of 
nervous  disturbances,  and  more  frequently  than  other  condi- 
tions constitute  a  neuropathic  tendency." 

The  Commission  was  composed  of  seven  medical  men  in 
addition  to  Dr.  Stevens,  and  they  investigated  the  results  of 
Stephens'  treatment  by  glasses  or  operation,  all  other  thera- 
peutic measures  being  excluded.  Their  finding  was,  that  so  far 
as  their  investigations  warranted  a  conclusion,  the  methods  used 
did  not  afford  a  sufficient  degree  of  relief  to  patients  suffering 
from  chorea  or  epilepsy,  to  justify  its  adoption  or  recommenda- 
tion as  a  means  of  cure,  or  as  a  sole  therapeutic  measure. 

Starr.2  says  it  is  important  in  nervous  disease  to  ascertain 
whether  or  not  any  form  of  peripheral  irritation  is  present,  but 
unless  it  is  serious,  and  has  produced  discomfort  which  has  been 
spontaneously  observed  by  the  patient,  not  much  importance 
is  to  be  attached  to  it  as  an  setiological  factor.  He  does  not 
believe  that  true  epilepsy  or  chorea  can  be  produced  by  eye 
strain  or  cured  by  its  relief. 

On  the  other  hand,  Wigglesworth  and  Bickerton  3  came  to 
the  conclusion  from  the  examination  of  103  epileptics,  that 
"  certain  cases  of  epilepsy  do  occur  in  which  the  attack  is 
induced  by  the  undue  strain  put  upon  the  muscular  apparatus 
of  the  eye,  by  reason  of  an  abnormality  of  refraction." 

The  point  I  think  does  not  need  labouring,  that  if  in  these 

1  Jour,  of  Nervous  and  Mental  Dis.,  1889,  633. 

2  Medical  Record,  1890,  xxxvii.,  3. 
s  Brain,  1888,  xi.,  479. 


104  OPI1THALMOPLEGIA   EXTERNA 

patients  a  refractive  error  is  present,  which,  however,  is  not 
measured  in  one-eighth  of  dioptres,  nothing  but  good  will  come 
of  its  correction  ;  but  there  seems  sufficient  evidence  to  make  it 
certain,  that  errors  of  refraction  or  of  the  muscular  balance  of 
the  eyes  are  at  most  of  trivial  importance  in  the  aetiology  of 
epilepsy  or  chorea. 

OPHTHALMOPLEGIA   EXTERNA. 

Paralysis  of  the  external  muscles  of  the  eyes  may  result 
from  : — 

(a)  Intra-cranial  disease,  e.g.,  tumours,  meningitis,  cerebral 
syphilis,  haemorrhage,  polio-encephalitis,  hydrocephalus,  pres- 
sure of  aneurysms  or  diseased  arteries,  thrombosis  of,  or  arterio- 
venous  aneurysms  in  the  cavernous  sinus,  etc. 

(6)  Disease  in  the  orbit  as  by  involvement  in  growths,  or  by 
acute  infections  of  the  tissues,  abscess  or  cellulitis. 

(c)  Trauma,  as  in  fractures  of  the  skull,  etc.     Toxins,  as  in 
diphtheria  and  perhaps  syphilis,  diabetes,  and  lead. 

(d)  It  may  occur  as  a  congenital  and  hereditary  affection. 

(e)  And  lastly  in  a  few  cases  no  satisfactory  cause  is  dis- 
covered, and  these  are  often  attributed  to  cold  or  to  rheumatism. 

In  some  cases  parts  only  of  a  nerve  are  paralysed,  the  other 
parts  escaping  ;  thus,  in  the  case  of  the  oculo-motorius,  the 
levator  palpebrse,  superior  rectus,  or  internal  muscles  may 
alone  be  affected,  indicating  in  most  cases  that  the  nucleus  of 
the  nerve  is  involved,  as  in  tabes,  or  polio-encephalitis. 

That,  however,  a  partial  symmetrical  paralysis  which  would 
suggest  a  nuclear  lesion  may  result  from  a  lesion  of  the  nerves 
themselves,  is  shown  by  Thomsen's  case,1  in  which  impaired 
upward  movement  of  both  eyes  resulted  from  a  gumma  which 
involved  one  third  nerve  and  pressed  upon  the  other. 

Diplopia  is  a  usual  accompaniment  of  these  palsies,  and 
except  in  the  congenital  cases  is  usually  confusing  to  the 
patient,  so  much  so,  that  he  will  frequently  elect  to  keep  one 
eye  completely  covered. 

In  cases  of  long  standing,  however,  the  false  image  of  the 

1  Cenlralb.  f.  Augenhcilk.,  1886,  203. 


DJPLOPIA  105 

squinting  eye  comes  to  be  ignored,  diplopia  is  then  no  longer 
troublesome,  and  the  patient  is  able  to  walk  about  without 
difficulty  with  both  eyes  uncovered. 

In  such  diseases  as  insular  sclerosis  and  myasthenia  gravis, 
it  is  not  uncommon  for  an  intermittent  diplopia  to  be  complained 
of,  which  no  doubt  indicates  an  incipient  defect,  but  is  not 
accompanied  by  any  obvious  limitation  of  movement  of  the  eyes. 

And  similarly,  after  the  recovery  of  a  paralysis,  diplopia, 
easily  elicited  and  sometimes  manifesting  itself  spontaneously, 
remains  long  after  any  defective  movement  of  the  eye  is 
discernible.  (See  also  p.  125.) 

Where  a  muscle  is  paralysed,  an  undue  effort  put  forth  on 
the  part  of  the  patient  in  an  endeavour  to  put  the  weakened 
muscle  into  action,  is  also  conveyed  to  its  synergic  muscle,  with 
the  result  that  in  it  an  over-action  is  produced,  and  a  secondary 
squint  occurs  ;  for  the  same  reason,  the  position  in  space  of  the 
object  producing  the  true  image  is  falsely  estimated. 

It  often  happens  that  diplopia  is  limited  to  that  part  of  the 
field  of  vision  towards  which  the  eye  would  be  moved  by  the 
paralysed  muscle,  and  the  patient  endeavours,  by  keeping  his 
head  in  a  certain  position,  to  use  that  portion  only  of  the  visual 
field  in  which  diplopia  does  not  occur.  Thus,  for  instance,  if 
the  right  external  rectus  muscle  is  paralysed,  this  eye  tends  to 
turn  inwards,  and  if  the  head  and  eyes  are  directed  forwards 
double  vision  is  present ;  if,  however,  the  inward  divergence  is 
compensated  by  turning  the  head  laterally  to  the  right,  the  eyes 
can  now  be  directed  straight  forwards  without  diplopia  result- 
ing. This  lateral  rotation  of  the  head  is  usually  about  20 
degrees,  is  unconsciously  assumed,  and  is  very  characteristic. 
A  good  example  of  the  same  compensatory  phenomenon  is 
referred  to  under  the  head  of  ocular  torticollis. 

It  is  a  notoriouVfact,  that  paralysis  of  the  ocular  nerves  is 
of  uncertain  value  as  a  localising  sign  in  cerebral  disease,  and 
this  is  especially  true  of  sixth  nerve  palsies  (see  p.  42). 

Thus,  while  the  nerves,  or  their  nuclei,  or  the  higher  co- 
ordinating centres  may  be  involved  in  a  growth,  and  their 
paralysis  would  have  a  localising  value,  in  other  examples  the 
nerves  on  one  or  both  sides  may  be  effected  by  a  basal 


106  OPHTHALMOPLEGIA   EXTERNA 

meningitis,  or  their  conducting  power  impared  by  the  presence 
of  a  tumour,  which  yet  does  not  directly  affect  the  nerve  or 
its  connections ;  or  again  oedema  of  the  brain  or  serous 
meningitis  is  apparently  a  sufficient  exciting  cause  of  a  sign 
which  must  always  raise  apprehension  of  the  more  serious 
disease. 

Uhthoff  l  says  :-— 

(1)  A  complete  bilateral  sixth  nerve  paralysis  indicates  most 

probably  a  basal  origin. 

(2)  Bilateral  but  incomplete  paralysis  may  be  due  to  tumours 

within  the  brain  substance. 

(3)  Unilateral  paralysis  does  not  justify  the  diagnosis  of  a 

tumour  in  the  same  hemisphere. 

It  was  shown  by  Mierzejewski  and  Rosenbach  2  that  a  lesion 
of  one  side  of  the  pons,  just  above  the  nucleus  of  the  sixth 
nerve,  causes  a  loss  of  movement  of  both  eyes  towards  the  side 
of  the  lesion. 

It  is  believed  that  the  sixth  nucleus  is  connected  with  the 
third  nerve  nucleus  by  means  of  the  superior  longitudinal 
bundle,  and  that  the  cells  of  the  sixth  nucleus  produce  lateral 
movement  of  both  eyes  towards  its  side,  by  stimulating  not 
only  the  sixth  nerve,  but  also  the  third  nerve  of  the  opposite 
side,  through  the  intermediation  of  this  bundle. 

Having  regard  to  the  foregoing,  Gowers  3  differentiates  three 
types  of  sixth  nerve  paralysis  in  intra-pontine  lesions  :— 

(1)  The  external  rectus  is  paralysed,  the  opposite  internal 

rectus  being  intact. 

Here  the  sixth  nerve  fibres  are  interrupted  peripheral- 
wards  of  its  nucleus,  the  nucleus  being  unaffected ; 
impulses  to  the  opposite  internal  rectus  through  the 
superior  longitudinal  bundle  are,  therefore,  unin- 
terrupted. 

(2)  The  sixth  nucleus,  and  with  it  often  the  fibres  of  the 

1  Bowman  Lecture,  Trans.  Ophth.  Soc.  U.K.,  1914,  xxxiv,  70. 

"  Neurologisches  Centralblatt,"  1885,  iv.,  363. 
3  Trans.  Ophth.  Soc.  U.K.,  1887,  vii.,  269. 


SIXTH  NERVE  PALSIES  107 

seventh  nerve,  where  they  curve  over  the  nucleus — is 
affected. 

In  this  case  neither  eye  can  be  turned  towards  the 
lesion,  for  no  impulse  can  be  transmitted  either  to  the 
sixth  nerve,  or  to  the  opposite  third  nerve  through 
the  superior  longitudinal  bundle. 

(3)  There  is  loss  of  movement  of  both  eyes  towards  the 
lesion,  but  not  complete  palsy  of  the  external  rectus. 

The  eye  cannot  be  moved  beyond  the  middle  line,  but 
it  does  not  deviate  inwards. 

In  this  case  there  is  no  disease  of  the  sixth  nucleus, 
but  the  lesion  is  in  the  tegmentum  of  the  pons,  damaging 
the  centre  or  path  for  conjugate  movements. 

Paralysis  of  the  third  nerve  may  be  "distant "  in  its  causa- 
tion, though  less  often  than  paralysis  of  the  sixth,  thus : — 

Uhthoff  reports  two  cases  of  single  oculo-motor  paralysis; 
in  which  post-mortem  there  was  no  sort  of  direct  involvement 
of  the  nerve  or  nucleus.  He  says,  "  a  unilateral  isolated  com- 
plete third  nerve  paralysis  may  therefore  be  sometimes  caused 
by  pressure  from  a  distance,  whereas  bilateral  paralysis,  an 
association  with  paralysis  of  the  crossed  half  of  the  body,  or 
simultaneous  paralysis  of  other  cerebral  nerves,  afford  safe 
indications  of  a  basal  origin." 

Further,  he  states  that  paralysis  of  single  branches  of  the 
nerve,  especially  that  going  to  the  levator  palpebrae,  more  often 
indicates  a  lesion  of  the  nerve  through  pressure  from  a  distance, 
than  involvement  of  the  nerve  trunk  or  cerebral  cortex. 

Owing  to  the  close  proximity  of  the  two  third  nuclei  to  each 
other,  and  also  of  the  nerves  where  they  leave  the  brain  in 
the  interpeduncular  space,  a  single  lesion,  either  in  the  latter 
situation,  or  in  the  region  of  the  corpora  quadrigemina  may 
involve  both  merves.  Goldzieher  l  reports  a  case  in  which 
there  was  paralysis  of  all  the  muscles  supplied  by  both  third 
nerves,  associated  with  weakness  of  the  lower  limbs  and  a 
staggering  gait.  A  tuberculous  tumour  the  size  of  a  hazel  nut 
was  found  post-mortem  just  under  the  corpora  quadrigemina. 

1  Centralbalt  fur  Augenheilk.,  1893,  44. 


108 

He  concludes,  that  ophthalmoplegia  with  inco -ordination  of 
the  lower  limbs  points  to  a  lesion  of  the  corpora  quadrigemina. 

Bruce  x  reports  a  case  in  which  upward  movement  of  the 
eyes  was  completely  absent,  downward  movements  were  im- 
perfect, but  lateral  movements  were  complete  ;  there  was  a 
tendency  for  the  patient  to  fall  backwards  when  she  shut  her 
eyes  ;  paralysis  of  the  elevators  of  the  lids  developed  one  day 
only  before  death.  At  the  post-mortem  a  glioma  was  found, 
"  extending  from  a  point  midway  between  the  two  corpora 
quadrigemina,  as  high  as  the  third  ventricle,  into  the  posterior 
part  of  which  it  projected  like  half  a  bean."  The  anterior  part 
of  the  third  nucleus  was  involved,  but  its  posterior  part  and 
the  nucleus  of  the  fourth  nerve  had  entirely  escaped. 

Gowers  2  says  :  "  Paralysis  of  the  upward  movement  of  the 
eyes  has  often  been  observed  associated  with  paralysis  of  the 
levators.  It  may  result  from  disease  of  the  posterior  part  of 
the  third  nerve  nucleus.  It  is  possible  also  that  there  is  a 
higher  centre,  disease  of  which  may  paralyse  upward  move- 
ments without  the  lid.  I  have  recorded  one  such  case  in  which 
this  symptom  was  well  marked.  A  very  small  tumour  was 
found  in  the  mid -line  behind  the  posterior  quadrigeminal  bodies , 
damaging  these  slightly,  the  velum,  and  the  adjacent  part  of 
the  inferior  vermis  of  the  cerebellum." 

Ferrier  3  gives  the  following  as  symptoms  of  involvement  of 
the  corpora  quadrigemina  :— 

A  markedly  reeling  gait,  which  most  often  develops  before 
ocular  symptoms. 

Ptosis,  limitation  of  vertical  eye  movements,  and  limitation 
of  lateral  eye  movements  in  this  order. 

He  states  that  there  is  no  evidence  that  the  anterior  corpora 
quadrigemina  are  concerned  with  visual  sensation,  and  that  the 
ocular  symptoms  are  due  to  the  involvement  of  the  underlying 
oculo -motor  nuclei. 

Aldren  Turner  likewise  remarks  that  removal  of  the  quad- 
rigeminal bodies  in  monkeys  does  not  cause  ocular  paralysis, 

1  Trans.  Endinburgh  Medico-CMr.  Soc.,  1899,  xix.,  206. 

"  Diseases  of  the  Nervous  System,"  1893,  ii.,  185. 
3  "  Allbutt  and  Rolleston,"  Syst.  of  Met!.,  1910,  viii.,  120. 


MID-BRAIN  LESIONS 


109 


if  care  is  exercised  to  avoid  damaging  the  grey  matter  around 
the  Sylvian  aqueduct. 

It  would  seem,  then,  that  the  association  of  a  reeling  gait 
with  oculo-motor  palsies,  in  which  the  muscles  producing 
vertical  movements  are  primarily  involved,  is  suggestive  of  a 
lesion  in  the  region  of  the  corpora  quadrigemina. 

Gordon  Holmes  l  believes  that  a  lesion  of  the  anterior  end 
of  the  mid-brain  will  involve  upward  movements,  downward 
movements,  and  convergence,  in  this  order  as  it  progresses  from 
before  back,  and  states  that  paralysis  of  the  pupil  is  often 
associated  with  loss  of  upward  movement,  and  loss  of  con- 
vergence with  paralysis  of  downward  movement. 

As  in  the  case  of  conjugate  lateral  movements,  it  is  probable 
that  there  is  a  superanuclear  centre  to  control  conjugate  vertical 
movements,  but  its  site  is  not  accurately  known. 

Congenital  and  hereditary  paralysis  of  the  ocular  muscles 
are  common,  and  many  pedigrees  are  fully  reported.  In  some 
the  external  recti  alone  are  involved,  in  others  there  is  ptosis, 
and  in  others  again  there  is  complete  ophthalmoplegia  externa, 
associated  in  a  few  cases  with  facial  paralysis.2 

Chronic  Progressive  Ophthalmoplegia  Externa. — Under  this 
title  may  be  classed  a  group  of  cases  which  was  first  identified 
by  Wilbrand  and  Saenger.3 

The  characteristics  are  :— 

( 1 )  A  slowly  progressive  lesion  which  is  bilateral,  and  which 

in  most  cases,  ends  ultimately  in  complete  or  nearly 
complete  ophthalmoplegia  externa. 

(2)  The  onset  is  usually  in  early  life  and  is  gradual. 

(3)  It  is  unassociated  with  any  other  morbid  condition  of  the 

nervous  system  and  does  not  shorten  life. 

Ptosis  is  usually  the  first^sign. 

Hine  and  McMullen,4  in  reporting  three  cases,  state  that  in 

six  only  out  of  thirty-eight  reported  cases  was  the  age  at  onset 

1  Trans.  Roy.  Soc.  Med.,  Neurol.  and  Ophth.  Sec.,  March,  1921. 

2  Bradbourne,    Trans.    Ophth.  Soc.   U.K.,  1912,   xxxii.,    142  ;    Harlan,  Trans. 
Amer.  Ophth.  Soc..,  1880,  iii.,  216.  and  iv.,  48  ;  Berry,  Ophth.  Review,  1886,  v.,  34  ; 
Xettleship,  Trans.  Ophth.  Soc.  U.K.,  1909,  xxix.,  clxxxviii.,  Bowman  Lecture. 

3  "  Neurologic  des  Auges,"  Wiesbaden,  1900. 

*  Trans.  Royal  Soc.  Med.,  Ophth.  and  Neurol.  Sections,  March,  1921. 


110  OPHTHALMOPLEGIA   IN  TERN  A 

over  thirty  ;  they  say  the  condition  is  attributed  to  an  abio- 
trophy  of  the  cells  of  the  third  nerve  nucleus,  the  time  of  onset 
depending  on  the  degree  of  vitality  with  which  the  cells  were 
originally  endowed. 

OPHTHALMOPLEGIA   INTERNA. 

Every  now  and  then  a  patient  is  seen  with  unilateral  ophthal- 
moplegia  interna,  which  is  apparently  permanent,  and  for  which 
there  is  no  discoverable  cause  either  at  the  time  or  ultimately. 
I  have  seen  a  patient  in  whom  it  was  known  to  have  been  present 
for  twenty -four  years  without  alteration  in  any  way. 

Uhthoff  collected  thirty  cases  of  ophthalmoplegia  interna,  in 
twelve  of  which  no  cause  was  discovered.  They  were  all  young 
and  were  said  to  be  of  a  neuropathic  disposition  ;  in  most  of 
them  the  symptoms  were  of  short  duration,  but  in  one  they  had 
been  present  without  change  for  twenty  years.  He  suggests 
that  some  such  cases  may  be  due  to  ptomaine  poisoning. 

Similar  cases  are  reported  by  v.  Hippel,1  who  states  his 
patients  had  neuropathic  tendencies  ;  Levinsohn 2  ;  and 
Bramwell  and  Sinclair. 

In  addition,  it  is  not  very  rare  to  come  across  pupils  which 
are  quite  inactive  to  light  stimulus,  whether  direct  or  consensual, 
but  which  contract  slowly  with  accommodation,  taking  some 
seconds  to  attain  complete  contraction,  and  are  as  slow  in 
again  relaxing. 

They  are  unilateral  ;  the  function  of  accommodation  is  quite 
unimpaired. 

They  are  unrelated  to  syphilis  or  any  general  nervous 
disorder.  I  have  notes  of  five  cases,  in  one  of  which  the 
condition  was  known  to  have  been  present  for  more  than  forty 
years,  and  in  another  for  more  than  twenty. 

The  features  lire  the  unilaterality,  the  intact  accommodation, 
the  tardiness  of  the  reaction  with  accommodation,  the  perma- 
nency of  the  condition,  the  absence  of  any  general  nervous 
disorder,  and  the  absence  of  syphilis. 

1  Klin.  Monals.f.  Aug.,  1906,  97. 

2  Archiv.  of  Ophlh.,  1901,  166. 


ENCEPHALITIS  LETHARGIC  A  111 

HYDROCEPHALUS. 

Owing  to  the  bulging  downwards  of  its  roof,  the  orbital  cavity 
is  a  good  deal  distorted  in  this  condition  ;  the  eyes  consequently 
become  rotated  downwards  and  are  largely  covered  by  the 
lower  lids ;  the  upper  lids  tend  to  be  drawn  upwards  by  the 
tightness  of  the  scalp. 

Still  states  that  in  some  cases  the  children  acquire  the  habit 
of  drawing  down  their  own  lower  lids  in  order  to  see. 

Any  prominence  of  the  eyes  is  masked  by  the  bulging  frontal 
region,  and  marked  proptosis  is  rare. 

Defects  of  vision  are  common,  but  good  vision  may  be 
retained  though  the  head  is  greatly  enlarged. 

All .  observers  are  agreed  that  these  defects  depend  upon  a 
primary  optic  atrophy  and  that  papilloedema  does  not  occur. 

Thus,  Gushing  l  says  :  "A  pressure  sufficient  to  produce  an 
advanced  degree  of  choked  disc  cannot  well  occur,  as  the 
cranium  is  so  easily  distensible." 

Still  says  :  "  Optic  neuritis  is  almost  never  seen  with  congenital 
hydrocephalus  ;  its  presence  always  points  to  cerebral  tumour 
as  the  cause  of  the  hydrocephalus  "  ;  and  Gowers  2 :  "In  several 
cases  the  onset  of  the  atrophy  has  been  watched,  and  the 
occurrence  of  any  neuritic  process  excluded." 

Batten  3.  says  :  "  Nystagmus  is  frequent  and  may  be  lateral 
vertical  or  rotatory  ;  squint  from  ocular  paralyses  is  often 
present." 

ENCEPHALITIS   LETHARGICA. 

This  epidemic  disease  to  which  various  titles  have  been 
applied,  has  come  into  much  prominence  in  Europe  and  America 
during  the  last  three  years. 

Paralysis  of  the  cranial  nerves  and  especially  of  the  ocular 
nerves  is  one  of  its  prominent  features  ;  so  much  is  this  the 
case  that  "  Acute  Infectious  Ophthalmoplegia  "  and  "  Toxic 
Ophthalmoplegia  "  are  amongst  the  names  which  have  been 
applied  to  it. 

1  Osier  and  McCrae's  "  Sys.  of  Med.,"  vol.  v. 

2  "  Med.  Ophthal.,"  160. 

3  Garrod  and  Thursfield  "  Diseases  of  Children,"  1913,  817 


112  ENCEPHALITIS  LETHARGIC  A 

The  other  prominent  clinical  features  are  those  of  an  acute 
general  infection,  associated  with  apathy  passing  into  lethargy, 
and  great  muscular  debility. 

The  ocular  symptoms  consist  of  paresis  or  paralysis  of  some 
of  the  ocular  nerves,  nystagmus,  photophobia,  and,  in  a  few 
cases,  changes  in  the  optic  discs.  It  is  a  feature  of  the  paralyses 
that  they  are  often  evanescent  or  intermittent  and  incomplete. 
Thus  it  is  common  for  certain  muscles  which  are  supplied  by 
the  third  nerve  to  be  involved,  the  others  escaping  ;  the  affected 
muscles  are  often  only  partly  paralysed  ;  ptosis^is  the  most 
common  partial  manifestation. 

Diplopia  may  be  a  very  early  prodromal  sign  which  quickly 
disappears  as  the  disease  becomes  established  ;  this  was  the 
case  in  20  per  cent,  of  the  168  cases  investigated  by  the  Local 
Government  Board,  London.1  It  may  afterwards  recur  and 
become  protracted. 

Of  the  above  cases  75  per  cent,  had  paresis  or  paralysis 
involving  the  third  nerve,  and  of  ninety-seven  cases  which  I 
have  collected  2  75  per  cent,  had  some  ocular  palsy. 

Ptosis  is  the  most  common  defect  and  in  25  per  cent,  of  the 
Local  Government  Board  cases  it  was  unilateral. 

In  27  per  cent,  of  my  collected  cases  the  sixth  nerve  was 
involved,  but  some  of  the  cases  labelled  diplopia  should  be 
added  to  this  figure. 

Various  changes  in  the  pupils  have  been  noted,  but  there  does 
not  appear  to  be  any  characteristic  feature  of  them,  and  involve- 
ment of  the  internal  branches  of  the  third  nerve  is  very  unusual  ; 
it  is  mentioned  in  three  of  my  ninety-seven  cases. 

Papillcedema  is  rare  ;  it  is,  mentioned  in  four  of  the  above 
cases.  The  Local  Government  Board  Report  says  :  "  Optic 
neuritis  has  been  seen  exceptionally.  Its  occurrence,  however, 
seems  to  be  limited  to  cases  in  which  hsemorrhagic  infarction 

1  Report  of  the  Local  Government  Board  on  Encephalitis  Lethargica,  No.  121, 
London, 1918. 

2  Ely,  Jour.  Amcr.  Med.  Ass.,  Ixxii.,  985  ;  Pothner.  Jour.  Amer.  Med.  Ass.,  Ixxii., 
715;  Tucker,  Jour.  A mer.  Med.  Ass.,  Ixxii.,   1448;   Wegeforth  and  Ayer,  ./«/'/. 
Amer.  Mad.  Ass.,  Ixxiii.,  5 ;   Kinnier-Wilson,  Lancet,  1918,  ii.,  7;    Cordon,  ,\ .  )  . 
Med.  Jour.,  cix.,  837  ;     Sachs,  X.  Y.  Med.  Jour.,  cix.,  894  ;     Hall,  B.M.J.,  1918, 
ii.,  461  ;     Melland,  B.M.J.,  1918,  i.,  559  ;    Dunn  and  Heagey,  Amer.  Jour.  Med. 
Sci.,  cix.,  568  ;  Netter,  Bull,  et  Mem.  #oc.  Med.  des  Hopitaux  de  Paris,  xlii.,  307. 


OCULAR  PALSIES 


113 


into  the  brain  substance  has  taken  place.  This  gives  rhe  to 
general  cerebral  hyper-tension  with  consequent  optic  neuritis/ 

Of  seventeen  cases  which  I  have  examined  ophthalmosco- 
pically,  in  no  case  did  the  fundi  show  any  change  which  could 
be  referred  to  the  disease. 

Nystagmus  was  mentioned  in  9  per  cent,  of  the  ninety -seven 
cases. 

Ocular  palsies  are  frequently  the  first  symptom  of  the  disease, 


FIG.  36. — A  section  through  the  abducens  nucleus  from  a  case  of  "  epi- 
demic encephalitis."  The  large  motor  cells  show  changes  varying 
fron;  intense  cloudy  swelling  to  a  moderate  degree  of  chromatolysis, 
in  a  few  instances  the  cell  bodies  showed  vacuolisation.  There  were 
numerous  small  haemorrhages  apparently  in  relation  to  the  veins,  all 
of  which  were  intensely  injected.  (Tilney  and  Howe,  "  Epidemic 
Encephalitis,"  New  York,  1920,  15.) 

and  so  also  they  may  be  the  last  to  disappear  after  the  patient 
is  convalescent ;  occasionally  they  are  permanent.  I  have 
seen  a  man  in  whom  residual  paralysis  of  both  third  nerves 
was  marked  twenty-one  months  after  the  onset  of  the  disease. 
Of  the  Local  Government  Board  cases,  twenty -six  were  examined 
at  various  times  after  recovery  ;  fourteen  of  them  still  had  ocular 
palsies  at  an  average  period  of  nine  weeks  after  the  onset  of  the 
disease. 


114  TABES  AND  GENERAL  PARALYSIS 

Pathological  evidence  shows  that  the  most  advanced  changes 
are  to  be  found  in  the  mesencephalon  in  the  region  of  the  third 
nerve  nucleus. 

Tilney  and  Howe  l  describe  the  degenerative  changes  in  the 
cells  of  the  nuclei  of  the  nerves,  cellular  infiltration,  vascular 
engorgement  and  small  hsemorrhagic  areas.  Of  the  sixth  nerve 
nucleus  (Fig.  36),  they  say  :— 

"  The  large  motor  cells  showed  evidence  of  acute  change, 
varying  from  intense  cloudy  swelling  to  a  moderate  degree  of 
chromatolysis.  In  a  few  instances  the  cell  bodies  showed 
vacuolisation." 

The  vascular  picture  was  similar  to  that  observed  in  the 
medulla,  and  was  characterised  by  numerous  small  haemorrhages 
apparently  in  relation  to  the  veins,  all  of  which  were  intensely 
injected. 

POLIO-ENCEPHALITIS. 

The  occurrence  of  a  paralytic  internal  squint  in  young 
children  with  a  history  of  some  recent  infectious  disease  is  often 
due  to  acute  polio -encephalitis, 

Stephenson  reports  twenty-eight  cases  in  three-quarters  of 
which  the  external  rectus  was  involved. 

Batten  points  out  that  they  are  often  associated  with  a 
rhythmic  tremor  of  the  limbs,  and  some  other  cranial  nerve 
palsies,2  and  Holden 3  remarks  that  the  usual  absence  of 
involvement  of  the  intrinsic  eye  muscles  is  a  distinctive  feature 
of  some  differential  value. 

A  case  in  which  almost  complete  recovery  of  movement 
occurred  in  a  month  is  reported  by  Greeves.4 

THE  INTRA-OCULAR  LESIONS  IN  TABES   AND 
GENERAL  PARALYSIS. 

The  Argyll  Robertson  pupil  is  almost  invariably  of  syphilitic 
origin,  either  acquired  or  congenital,  and  rarely  occurs  apart  from 
tabes  or  general  paralysis  ;  it  is,  however,  true  that  the  pupils 

1  "  Epidemic  Encephalitis,"  Paul  Hoeber,  New  York,  1920. 

2  Lancet,  1916,  809. 

*  Trans.  Amer.  Ophih.  Soc.,  1906-8,  xi.,  220. 
4  Trans.  Ophih.  Soc.  U.K.,  1912,  xxxii.,  104. 


115 

'of  a  patient  who  is  blind  will  usually  contract  with  convergence, 
though  they  are  inactive  to  light  stimulus. 

Gowers  comments  on  the  absence  of  pupillary  changes  in 
other  spinal  diseases  in  which  Rombergism,  ataxy,  and  changes 
in  the  knee  jerks  may  all  occur,  and  especially  on  their  absence 
in  combined  sclerosis  of  the  lateral  and  posterior  columns. 

The  actual  site  of  the  responsible  lesion  cannot  be  considered 
as  definitely  settled.  Bumke's  1  great  weight  and  authority 
in  the  matter,  however,  locate  it  in  the  terminal  ramifications 
of  the  centripetal  fibres  from  the  retina  in  the  neighbourhood 
of  the  iris  sphincter  centre.  By  some  the  lesion  is  placed  in 
the  cells  of  the  partial  nucleus  of  the  third  nerve,2  and  by  others 
in  the  ciliary  ganglion. 

Wilfred  Harris 3  suggests  that,  in  conformity  with  the 
degenerations  in  the  posterior  columns  of  the  cord,  the  lesion  is 
more  likely  to  be  in  nerve  fibres  than  in  cells.  He  points  out 
that  in  birds  and  some  lower  animals  there  is  a  complete  decus- 
sation  of  the  optic  nerves,  and  that  in  them  the  consensual  pupil 
reaction  does  not  occur  ;  there  must,  therefore,  be  a  complete 
posterior  decussation  between  the  optic  lobes  and  the  third 
nerve  nucleus.  He  argues  similarly  that  in  man,  with  binocular 
vision  and  a  semi-decussation  of  the  optic  nerves,  a  correspond- 
ing posterior  semi-decussation  holds  good  between  the  anterior 
corpora  quadrigemina  and  the  oculo-motor  nucleus,  which  sub- 
serve^ the  light  reflex.  This  semi-decussation  he  locates  in 
MynertVbundle  which  goes  from  one  anterior  corpus  quadri- 
geminum  to  both  third  nerve  nuclei,  and  he  suggests  that  the 
lesion  in  the  Argyll  Robertson  pupil  is  a  sclerosis  of  one  or  other 
of  these  bundles,  according  as  the  loss  of  light  reflex  is  uni-  or 
bi-lateral. 

Incidence. — Of  '^eventy-seven  cases  of  tabes  examined  by 
Rochon  Duvigneaud  and  Heitz  4  in  four  only  could  the  pupil 
reaction  be  considered  normaj/  and  of  seventy-two  cases 
examined  by  Gowers  5  the  internal  muscles  of  the  eye  were 

1  "  Die  Pupillenstorungen  bei  s,eister  und  nerven  krankheiten,"  Jena,  1911. 

2  Sturge,  Trans.  Ophtk.  Soc.  U.K.,  1881,  i.,  177. 

3  B. M.  Jour.,  1900,  ii.,  924. 

*  Archiv.  gen.  de  Med.,  1903,  cxcii.,  1675. 
6  Trans.  Ophth.  Soc.  U.K.,  1883,  iii.,  198. 

8—1 


116          TABES  AND  GENERAL  PARALYSIS 

normal  in  six  only.  In  1,036  cases  of  general  paralysis  Jimius 
and  Arndt l  found  a  disturbance  of  the  light  reflex  in  93-8  per 
cent.,  and  in  65-8  per  cent,  it  was  totally  lost.  Bevan  Lewis2 
in  sixty  patients  found  impaired  reaction  to  light  in  78  per 
cent.  \^ 

It  is  not  rare  to  find  oneplipil  only  affected  ;  thus  twenty -three 
of  Junius  and  Arndt's  cases  were  unilateral,  and  Oliver  3  notes 
a  similar  proportion. 

iln  30  per  cent,  of  Rochon  Duvigneaud  and  Heitz's  cases 
there  was  bilateral  diminution,  or  loss  of  reflex  contraction  with 
convergence,  and  in  13  per  cent,  it  was  unilateral. 

Cycloplegia. — This  was  present  in  six  out  of  Gowers'  seventy- 
two  patients,  and  in  two  accommodation  was  lost  on  one  side 
only,  although  the  light  reflex  was  absent  on  both  sides.  In 
15  per  cent,  of  Gowers'  cases  the  pupils  were  inactive  with 
convergence  as  well  as  to  light  stimulus,  and  in  most  of  these 
cycloplegia  was  also  present ;  in  two  of  them  it  was  unilateral. 
-""  Cycloplegia  is  seldom  present  with  a  small  pupil  ;  on  the 
other  hand,  if  the  pupil  is  large  and  the  vision  is  good  paralysis 
of  accommodation  will  often  be  found  to  be  present. 

A  variation  in  the  size  of  the  two  pupils  is  so  common  as  to  be 
usual,  and  they  are.  ofteit-nlieven  in  shape  ;  it  is  also  common  to 
find  that  they  dilate^vely  little  with  mydriatics. 

Under  the  head  of  a  "  springing  "pupil,"  Oppenheim  speaks 
of  the  association  of  alternate  dilatation  of  the  pupils  with 
inactivity  to  light  stimulus  and  says  it  is  a  symptom  or 
prodrome  of  tabes  or  general  paralysis. 

Optic  Atrophy. — Loss  of  sight  due  to  optic  atrophy  is  not 
infrequently  the  first  symptom  for  which  advice  is  sought,  it 
may,  however,  occur  late  in  the  disease  and  then  its  progress  is 
usually  slow.  Gowers 4  has  seen  optic  atrophy  precede  loco- 
motor  symptoms  in  one  case  by  twenty  years,  and  in  another 
by  fifteen,  but  he  states  it  has  never  been  proved  that  optic 
atrophy  precedes  loss  of  knee  jerks. 

Both  eyes  are  always  involved,  but  one  is  usually  affected 

1  Arch.  f.  Psych,  und  Nervenkr.,  1908,  xliv.,  2,  493. 

2  Trans.  Ophth.  Soc.  U.K.,  1883,  iii.,  204. 

3  Trans.  Amer.  Ophth.  Soc..,  1891,  vi.,  544. 

4  Train.  Ophtlt.  Soc.  U.K.,  1883,  iii.,  193. 


OPTIC  ATROPHY 


117 


before  the  other,  and  the  eye  which  first  is  bad  maintains  the 
lead  throughout ;  it  is,  however,  unusual  to  find  the  sight  of  one 
eye  badly  deteriorated  without  the  other  being  involved  in 
some  degree. 

Frequency.— Kurt,  Mendel,  and  Tobias1  found  thirty- six 
cases  of  marked  optic  atrophy  amongst  430  tabetics,  and 
( lowers  2  states  :  "  Of  the  last  400  cases  of  tabes  I  have  seen,  optic 
atrophy  existed  in  twenty-six  (6-5  per  cent.)." 


FIG.  37. — A  longitudinal  section  through  the  optic  nerve  in  a  case  of 
tabetic  atrophy.     A.  Retina.     B.  Sclerotic. 

Amongst  seventy-six  cases  of  progressive  optic  atrophy, 
excluding  post-papillitic  cases,  examined  by  Nettleship,  thirty- 
eight  were  undoubtedly  tabetic. 

The  Relation  of  Optic  Atrophy  to  Ataxia. — It  has  long  been 
recognised  that  in  tabetics  who  develop  optic  atrophy,  the  onset 
of  ataxia  is  usually  much  delayed. 

Thus  Gowers  says  :  "  When  atrophy  commences  in  the  early 
stages,  the  progressive  tendency  is  often  strong,  and  that  of  the 
spinal  disease  slight,  and  conversely,  when  the  atrophy  com- 

1  "  Die  Tabes  der  Frauen."  Berlin,  1912, 
8  "  Med.  Ophthal.,"  1904,  181, 


118  TABES  AND   GENERAL  PARALYSIS 

mences  later  in  the  disease,  after  the  alteration  in  the  gait  has 
become  well  marked,  the  tendency  of  the  atrophy  to  progress  is 
much  less  marked.  Buzzard  found  four  cases  only  of  ataxia 
amongst  fifteen  blind  patients.  Forster1  found  thirteen  cases 
of  optic  atrophy  amongst  twenty-seven  patients  in  the  pre- 
ataxic  stage,  whereas  he  found  six  only  amongst  fifty-eight 
patients  in  whom  ataxia  was  present ;  further,  he  found  that 
the  average  preataxic  period  was  four-and-a-half  years  where 
there  was  no  optic  atrophy,  but  that  when  optic  atrophy  was 
present  this  period  averaged  nine-and-a-half  years. 

Maloney  2  does  not  believe  that  this  partial  dissociation  of 
optic  atrophy  and  ataxia  is  indicative  that  the  morbid  process 
affects  more  especially  the  corresponding  parts  of  the  central 
nervous  system  in  the  respective  groups,  but  that  the  explana- 
tion is  to  be  found  in  the  fact  that  blindness  increases  co-ordina- 
tion and  so  mitigates  ataxia.  He  quotes  the  investigations  of 
Slinger  and  Horsley.  3  These  observers  showed  that  those  who 
are  blind,  whether  from  tabes  or  any  other  cause,  possess  a 
"  greater  muscular  and  arthrodial  sense  accuracy  "  ;  they  state  : 
'  ...  if  the  information  gained  by  sight  is  permanently  blotted 
out,  the  muscular  sense,  under  necessity  can,  by  education,  be 
brought  to  a  point  at  least  one -quarter  better  than  that  learnt 
by  normal  seeing  individuals." 

Maloney  concludes  that  neither  the  occurrence  of  optic 
atrophy  nor  of  blindness  retards  or  influences  the  evolution  of 
the  structural  changes  which  accompany  the  tabetic  process  in 
the  spinal  cord  or  elsewhere,  and  that  improvement  in  ataxia 
will  result  from  a  lack  of  vision  after  an  adequate  interval  has 
elapsed — 

(a)  When  inco-ordinating  lesions  remain  stationary  and  the 
mental  capacity  for  training  is  not  destroyed  by  cerebral 
disease. 

(6)  Even  if  the  structural  tabetic  lesions  leading  to  inco- 
ordination  extend,  provided  that  the  effect  of  this 

1  Quoted  by  Maloney. 

2  Jour,  of  Nervous  and  Mental  Diseases,  1913,  xl.,  573 
•'  Brain,  1906,  xxix.,  26. 


VISUAL   FIELDS  119 

extension  be  less  than  the  compensatory  influence  of  the 
blindness. 

Thus,  of  two  tabetics  at  any  stage  of  the  disease,  one  blind, 
the  other  seeing,  ceteris  paribus,  the  blind  has  the  greater 
tendency  to  persist  co-ordinate,  the  lesser  tendency  to  become 
ataxic. 

The  degree  of  this  compensatory  improvement  of  co-ordina- 
tion will,  ceteris  paribus,  tend  to  rise  with  the  duration  of  the 
blindness  according  to  the  training  capacity  of  the  person. 

Fields  of  Vision. — It  has  been  demonstrated  by  Fuchs l  that 
the  occurrence  of  a  central  scotoma  is  not  very  rare  in  tabes. 
At  first  the  scotoma  is  for  red  and  green,  and  involves  the 
yellow  spot  and  is  associated  with  an  enlargement  of  the  blind 
spot. 

Later  these  two  blind  areas  enlarge  and  coalesce  and 
the  loss  of  sight  over  them  is  absolute.  The  condition  is 
separated  from  toxic  amblyopia  by  its  malignant  course, 
by  the  contraction  of  the  peripheral  field,  and  by  the  fact 
that  colour  perception  is  interfered  with  throughout  the  field 
of  vision. 

With  regard  to  its  relation  to  the  general  disease,  Fuchs 
says  :  "  I  do  not  think  of  denying  that  cases  of  syphilitic  retro- 
bulbar  neuritis  do  exist,  but  I  affirm,  that  in  the  great  majority, 
the  central  scotoma  does  not  form  an  accidental  complication, 
but  an  integral  part  of  the  tabetic  process." 

Nettleship  pointed  out  that  the  last  part  of  the  visual 
field  to  disappear  was  often  in  the  lower  temporal  area — 
an  area,  it  may  be  noted,  which  is  the  last  part  to  disap- 
pear when  the  temporal  field  is  lost  in  pituitary  lesions,  and 
which  is  sometimes  spared  in  embolism  of  the  central  retinal 
artery. 

Uhthoff  estimated  the  frequency  of  a  central  scotoma  as 
about  2  per  cent.,  Galezowski  and  Lobel  2  at  from  2  to  4  per 
cent. 

On  six  occasions  Fuchs  has  ssen  bitemporal  hemianopia, 

1  Archiv.  of  Ophth.,  1911,  xl.,  469  ;  Trans.  Amer.  Ophth.  Soc.,  1909-11,  xii.,  718. 
3  Recuell  cTOphih.,  April,  1906. 


120  ACUTE  MYELITIS 

which  he  believes  to  be  an  essential  part  of  the  disease  in  such 
cases. 

Whilst  loss  of  impairment  of  colour  vision  is  very  common 
in  tabes,  Dodd  l  reports  a  case  in  which  the  patient  after 
having  for  some  years  failed  to  appreciate  red,  suddenly  saw 
everything  as  through  a  brilliant  green  curtain.  He  collects  2 
thirteen  cases  of  green  vision,  all  but  three  of  which  occurred 
with  disease  of  the  optic  nerve  or  retina. 

FRIEDREICH'S   ATAXIA. 

According  to  Friedreich's  original  account,  the  only  eye 
lesion  of  this  disease  is  nystagmus,  and  all  authors  agree  in 
considering  other  ocular  lesions  as  of  quite  unusual  occurrence. 

Nystagmus  is  exceedingly  common,  especially  in  advanced 
cases,  and  Posey  and  Spiller  3  say  that  only  very  rarely  is  it 
absent.  Gordon  Holmes,4  however,  whilst  agreeing  that  it  is 
common,  says  it  cannot  be  looked  upon  as  one  of  the  essential 
symptoms  of  the  disease. 

It  usually  occurs  only  on  volitional  movement,  and  especially 
in  the  horizontal  plane  ;  it  is  not  as  a  rule  present  when  the 
eyes  are  at  rest. 

Other  symptoms  which  have  been  reported  are  paralysis  of 
one  or  other  of  the  external  ocular  muscles,  diminished  reaction 
of  the  pupils  to  light,  reduction  in  the  visual  fields  and  optic 
atrophy. 

ACUTE  MYELITIS  WITH  RETRO-BULBAR  NEURITIS. 

Under  some  title  similar  to  the  above  a  number  of  cases 
of  a  disease  which  is  probably  a  clinical  entity,  have  been 
recorded. 

The  association  of  the  two  conditions  was  remarked  by  Sir 
Clifford  Allbutt,5  and  in  1914  Goulden  6  was  able  to  collect 

1  Trans.  Ophth.  Sec.  U.K.,  1899,  xix.,  281. 

2  Trans.  Ophth.  Soc.  U.K.,  1900,  xx.,  264. 
3,j"  The  Eye  and  Nervous  System,"  475. 

4  Allbutt  and  Rolleston's  Syst.  of  Med.,  1910,  vii  ,  775. 

6  Lancet,  1870,  i.,  77. 

6  Trans.  Ophth.  Soc.  U.K.,  1914,  xxxiv.,  229. 


OPTHALMOSCOPIC  CHANGES  121 

fifty -two  reported  cases.  The  disease  is  acutely  febrile,  and 
is  characterised  by  rapid,  severe  loss  of  sight,  and  symptoms 
of  acute  myelitis,  and  in  a  majority  of  cases  it  ends  fatally. 
In  four-fifths  of  the  cases  loss  of  sight  is  the  first  symptom. 
It  may  precede  the  myelitis  by  several  days  (Goulden)  ; 
it  is  rapid  and  may  be  complete  in  twenty -four  hours.1  In 
other  cases  the  eye  symptoms  follow  the  spinal  symptoms. 


FIG.  38. — "  Acute  myelitis  with  retro-bulbar  neuritis."  A  transverse  sec- 
tion through  the  spinal  cord  in  the  cervical  region,  showing  a  large 
patch  of  myelitis  in  the  dorsal  columns  of  the  cord.  (Gordon 
Holmes.) 

In  nineteen  out  of  twenty-one  cases  both  eyes  were  affected 
(Katz). 

The  ophthalmoscopic  changes  are  slight,  and  do  not  attain 
a  degree  to  which  the  term  papilloedema  is  applicable.  Other 
cranial  nerves  are  not  involved.  A  majority  of  cases  are  fatal 
within  a  short  time,2  but  if  the  patient  survives,  there  is  a 
tendency  towards  recovery  of  both  the  cord  and  eye  condition. 
The  lesions  are  probably  dependent  upon  a  common  bacterial 
toxin. 

1  Katz,  Archiv.  fur  Ophth.,  1896,  xlii.,  1,  202. 

2  Chisholm,  Archives  of  Ophth.,  1882,  239. 


122  DISSEMINATE  SCLEROSIS 

Complete  pathological  examinations  of  the  cord  and  optic 
nerves  have  been  made  by'Holden,1  Bielschowsky,2  Sharkey 
and  Lawford,3  Gordon  Holmes  4  (Fig.  38).  There  is  but  little 
divergence  in  the  pathological  findings  of  different  investigators. 
All  agree  that  the  lesions  are  strictly  focal.  There  are  localised 
areas  of  softening  and  necrosis,  with  considerable  perivascular 
lymphocytic  infiltration  (Gordon  Holmes),  and  neuroglial  over- 
growth (Holder).  Bielschowsky  shows  that  the  infiltration 
is  in  connection  with  individual  vessels.  The  myelin  sheaths 
are  completely  destroyed,  but  a  considerable  proportion  of 
the  axis  cylinders  persists,  though  many  are  of  irregular 
contour  (Gordon  Holmes).  Sharkey  found  acute  inflammation 
of  the  optic  disc  and  nerve,  the  chiasm  and  tract  being  less 
involved. 

DISSEMINATE   SCLEROSIS. 

This  is  a  disease  of  young  adults  ;  it  is  exceedingly  seldom,  if 
ever,  seen  in  children,  and  it  is  rare  for  it  to  come  to  notice  for 
the  first  time  after  the  age  of  forty.  Thus,  85  per  cent,  of 
Byrom  Bram well's  cases  occurred  between  the  ages  of  fifteen 
and  thirty-five  ;  and  twelve  only  out  of  100  cases  examined 
by  Uhthoff  were  under  twenty  years  of  age. 

A  number  of  important  ocular  lesions  occur,  and  as  they  are 
frequently  the  first  symptom  of  the  disease,  the  ophthalmic 
surgeon  often  sees  these  patients  in  the  first  instance. 

The  chief  of  the  defects  may  be  considered  under  the  heads 
of  changes  in  the  visual  tracts,  defects  of  the  fields  of  vision, 
and  defects  of  muscular  action. 

Ophthalmoscopic  Change. — By  far  the  most  frequent  and 
important  ophthalmoscopic  change  is  the  occurrence  of  vary- 
ing grades  of  optic  atrophy  ;  this  may  be  slight,  or  rarely 
marked,  but  complete  atrophy  of  both  discs  at  all  com- 
parable with  that  which  may  be  seen  in  tabes,  leading  to 
complete  blindness,  is  almost  if  not  quite  unknown. 

1  Trans.  Amer.  Ophth.  Soc.,  1909-11,  xii.,  728. 
"  Myelitis  und  Sehnervenentziindung,"  Karger,  Berlin,  1901. 

3  Trans.  Ophth.  8oc.  U.K.,  1884,  iv.,  232. 

4  Trans.  Ophth.  Soc.  U.K.,  1914,  xxxiv.,  24G. 


DISC   CHANGES  123 

In  this,  as  in  optic  atrophy  from  other  causes,  there  is  often 
a  marked  disproportion  between  the  colour  of  the  disc  and 
the  visual  defect,  the  sight  may  be  either  much  better  or  much 
worse  than  would  have  been  predicted  from  the  colour  of  the 
disc,  and  as  is  to  be  expected  from  the  pathology  of  disseminate 
sclerosis,  the  eyes  may  be  very  unequally  involved. 

In  a  majority  of  cases  the  atrophy  appears  to  be  a  simple 
degenerative  process  so  far  as  ophthalmoscopic  evidence  is 
concerned,  for  in  few  is  there  evidence  of  active  changes  in  the 
optic  papilla  to  which  the  term  papillcedema  or  optic  neuritis 
could  be  applied.  In  a  small  proportion,  however,  such  active 
changes  may  be  seen,  and  when  they  occur  they  are  probably 
indicative  of  an  area  of  active  degeneration  close  behind  the 
optic  nerve  entrance.  They  do  not  imitate  at  all  closely  the 
great  swelling  and  oedema  of  the  nerve  head  that  occurs  with 
a  cerebral  tumour,  to  which  the  term  papilloedema  is  so  appro- 
priately attached ;  nor  do  they  indicate  the  increase  of  intra- 
cranial  pressure  which  papilloedema  implies.  The  disc  is 
slightly  swollen  and  its  edges  are  hazy,  the  presence  of  haemor- 
rhages or  exudate  is  unusual ;  the  moderate  changes  are  short- 
lived and  may  leave  a  normal -looking  disc  on  their  disappear- 
ance, but  in  most  cases  a  varying  degree  of  atrophy  subse- 
quently develops.  The  more  common  cases  of  atrophy,  without 
evidence  at  any  time  of  active  change  in  the  disc  so  far  as  is 
known,  are  probably  due  to  a  patch  of  sclerosis  in  some  portion 
of  the  nerve  remote  from  the  globe. 

Three  cases  with  "  mild  optic  neuritis  "  are  reported  by 
Sharkey  and  Nettleship.1  Nettleship  says  :  2  "If  the  optic 
nerves  suffer  in  disseminate  sclerosis  they  seldom  suffer  pro- 
foundly, and  often  recover  partially,  and  that  the  process  is 
more  acute,  and  to  the  opthalmoscope  more  inflammatory 
than  in  tabetic  atrophy." 

Uhthoff  believes  that  ophthalmoscopic  evidence  of  active 
change  is  more  common  than  is  generally  believed,  the  neuritis 
running  a  rapid  course  and  leaving  no  evidence  of  its  previous 
existence. 

1  Trans.  Ophth.  Soc.  U.K.,  1883,  iii.,  225  antf  227- 
3  Ibid.,  254. 


124  DISSEMINATE   SCLEROSIS 

In  recording  three  cases  in  which  active  disc  changes  were 
present,  Paton  l  summarises  the  changes  thus  :— 

(1)  The  loss  of  sight  precedes  the  disc  changes. 

(2)  The  ophthalmoscopic  changes  are  of  very  short  duration. 

(3)  The  disc  may  show  no  trace  of  pathological  change  after 

the  swelling  has  subsided. 

(4)  The  recovery  of  the  vision  is  usually  synchronous  with 

the  subsidence  of  the  swelling. 


FIG.  39. — A  transverse  section  through  the  optic  nerve  a  short  distance 
behind  the  globe  extensively  involved  in  a  patch  of  sclerosis,  the  part 
around  the  central  vessels  being  as  usual  especially  affected. 
(Velter.) 

He  suggests  that  when  disc  changes  are  found  to  be  of  very 
short  duration,  disseminate  sclerosis  should  be  had  in  mind. 

Uhthoff  found  ophthalmoscopic  evidence  of  some  degree  of 
optic  atrophy  in  forty-five  out  of  100  cases  examined  by  him. 
Buzzard  found  pallor  of  the  discs  in  43  per  cent,  of  cases. 

The  Loss  of  Sight. — It  is  by  no  means  rare  for  loss  of  sight  to 
be  the  earliest  sign  of  the  disease  ;  such  cases  almost  invariably 
come  first  into  the  hands  of  the  ophthalmic  surgeon,  and  are 
labelled  retro-bulbar  neuritis,  the  cause  of  which  is  by  no  means 

*  Trans,  Ophth.  Soc,  U.K.,  1914,  xxxiv.,  257, 


OCULAR  PALSIES  125 

always  certain,  and  it  is  only  the  subsequent  history  and  pro- 
gress which  makes  its  nature  clear. 

The  defect  may  develop  gradually  or  may  be  rapid  or  sudden 
in  its  onset  ;  it  is  variable  in  its  degree,  is  frequently  unilateral, 
and  often  temporarily  disappears,  only  to  recur  at  a  later 
date  ;  alternate  loss  of  sight  with  recovery  may  recur  several 
times  before  marked  ophthalmoscopic  changes  are  seen,  and 
the  amblyopia  becomes  permanent. 

Of  sixteen  cases  of  retro-bulbar  neuritis,  Marx  l  found  six 
subsequently  developed  insular  sclerosis,  and  Gunn  2  found  that 
of  233  cases  of  primary  retro-bulbar  neuritis,  fifty-one  were  due 
to  this  disease. 

Retro-bulbar  neuritis  may  precede  all  other  symptoms  of 
insular  sclerosis  by  as  long  as  ten  years.3 

Muscular  Abnormalities. — Paralysis  or  paresis  of  one  or 
other  of  the  ocular  muscles  is  frequent,  and  as  in  tabes,  may  be 
the  first  sign  of  the  disease,  and  may  disappear. 

Diplopia  ensues,  but  is  often  fugitive  and  varies  much  in  its 
degree,  it  ha 5  not  the  stability,  nor  is  it  so  confusing,  as  the 
diplopia  which  arises  from  many  other  acquired  ocular  palsies. 
The  defective  movement  causing  the  double  vision  may  be  so 
slight  as  not  to  be  easily  identified.  The  nerve  most  often 
affected  is  the  abducent,  a  partial  ocular  motor  paralysis  being 
next  most  common. 

Kampherstein  4  found  ten  cases  of  muscular  defect  amongst 
thirty-seven  cases  of  insular  sclerosis. 

Nystagmus. — This  is  one  of  the  classical  signs,  and  is  of  very 
frequent  occurrence  in  some  stage  of  the  disease.  It  was 
present  in  fifty -eight  out  of  100  cases.5  It  may  be  present 
with  the  eyes  at  rest,  but  this  is  not  usual,  more  often  it  is 
elicited  on  asking  the  patient  to  look  towards  the  periphery 
in  one  or  other  direction,  especially  laterally  ;  in  addition, 
Edwin  Bramwell6  describes  as  nystagmoid,  movements  which 

1  Archiv.f.  Augen.,  1908,  lix.,  28. 

2  Lancet,  1904,  ii.,  412. 

3  Fleischer,  Die  Ophth.  Klinik,  x.,  19. 

4  Archiv.f.  Augen.,  1903,  xlix.,  41. 

5  Uhthoff,  "  Dissem.  Scler.,"  Hirchwald,  Berlin,  1889. 

6  Osier's  "  System  of  Medicine,1'  1915,  v.,  151. 


126  DISSEMINATE  SCLEROSIS 

occur  during  fixation,  but  which  rapidly  cease,  and  are  at  the 
same  time  frequently  irregular  in  rhythm  and  amplitude. 

If  nystagmus  is  looked  upon  as  a  form  of  the  intention 
tremor  which  is  so  common  in  disseminate  sclerosis,  then  the 
explanations  of  the  mode  of  its  production  are  the  same  as  those 
put  forward  for  the  intention  tremors. 

Thus  Charcot  says,  the  axis  cylinders  being  denuded  of  their 
myelin  sheaths,  offer  a  varying  resistance  to  the  passage  of 
impulses  at  different  parts  of  their  course,  the  impulses  in 
different  axis  cylinders  consequently  arrive  at  their  muscular 
endings  at  irregular  times,  and  produce  irregular  contraction 
in  the  muscle. 

Risien  Russell  l  says  :  "Of  all  the  possible  explanations 
which  have  been  offered  of  the  way  in  which  the  intention 
tremor  results,  none  appear  to  me  to  be  so  satisfactory,  as  that 
which  supposes  it  to  be  due  to  a  failure  of  synergic  action  of 
muscles  in  purposive  movements,  consequent  on  an  unequal 
degree  of  innervation  both  of  the  muscles  directly  concerned 
and  of  their  antagonists." 

Fields  of  Vision. — Changes  of  the  visual  fields  are  frequent, 
in  some  cases  central  vision  is  affected,  the  scotoma  being  either 
for  colours  only  or  sometimes  being  complete.  In  other  cases 
central  vision  is  unaffected,  but  the  peripheral  limits  of  the  field 
are  constricted,  usually  in  an  irregular  manner.  In  other  cases 
again,  the  two  foregoing  conditions  are  combined  in  varying 
degrees. 

Of  fifteen  cases,  Kampherstein  found  eight  belonged  to  the 
first  group,  four  to  the  second,  and  three  to  the  third  group. 
Uhthoff's  2  figures  are  in  close  agreement.  He  says  that  in 
one-half  of  all  cases  with  defects  of  the  visual  fields  there  is  a 
central  scotoma,  usually  bilateral,  often  relative,  with  no 
alteration  in  the  peripheral  field.  Irregular  peripheral  con- 
traction with  intact  central  vision  is  frequent  ;  peripheral 
contraction  combined  with  central  scotoma  is  rare  ;  in  a  few 
cases  regular  concentric  constriction  occurs. 

Pupillary  Abnormalities.— The  pupillary  changes  which  may 

1  Allbutt's  "  System  of  Med.,"  1910,  vii.,  830. 

2  "  Ophthalmoscope,"  1905,  431. 


PATHOLOGICAL   CHANGES  127 

occur  in  disseminate  sclerosis  are  dependent  upon,  and  vary 
with,  the  existence  of  local  changes  in  the  tracts  concerned 
in  the  pupillary  reflex,  and  are  in  no  way  distinctive  of  this 
disease. 

Pathological  Changes. — The  pathological  changes  which 
develop  may  occur  in  any  part  of  the  central  nervous  system 
and  in  the  peripheral  nerves.1  The  islands  of  sclerosis  arise 
in  an  entirely  indiscriminate  manner  throughout  the  nervous 


FIG.  40. — Section  through  the  optic  chiasma  showing  degenerative  areas 
in  a  case  of  disseminate  sclerosis. 

system,  and  thus  is  explained  the  varied  manifestations  and 
course  of  the  disease. 

The  sites  of  predilection  are,  according  to  Dawson,2  related  to 
(1)  the  terminal  ramifications  of  end  arteries,  (2)  to  areas  where 
much  glia  is  normally  present,  e.g.,  the  optic  chiasma  (Fig.  40). 
Dawson  found  that  the  optic  chiasma  and  one  or  both  optic 
nerves  were  affected  in  seven  out  of  eight  cases  in  which  they 
were  examined,  and  in  six  of  them  there  was  extensive  involve- 
ment of  the  optic  radiations  on  both  sides.  The  earliest  changes 

1  Tredgold,  Rev.  of  Neurol.  and  Psych.,  1904,  ii.,  497. 
*  Trans.  Roy.  Soc.  of  Edinburgh,  1916,  1.,  517. 


128  8  YRINGOM  YE  LI  A 

which  occur  are  in  the  myelin  sheaths  ;  these  first  undergo 
cloudy  swelling,  followed  by  fatty  degeneration,  and  later,  com- 
plete absorption  occurs,  leaving  the  axis  cylinder  completely 
bare.  For  a  long  time  the  denuded  axis  cylinders  may 
maintain  their  functions,  or  they  may  temporarily  be  thrown 
out  of  gear  to  recover  for  a  time  later  on,  but  ultimately 
they  become  irregularly  swollen,-  broken  up  and  absorbed. 
As  a  result  of  these  changes,  proliferation  of  the  neuroglia 
takes  place  producing  the  patches  of  sclerosis  ;  the  sclerosis  on 
this  view  is  a  secondary  development  (Tredgold). 

Uhthoff  on  the  other  hand  looks  upon  the  changes  in  the 
connective  tissues  as  primary,  and  the  destruction  of  the  nerve 
tissue  as  a  secondary  result  of  this.  As  a  result  of  the  complete 
interruption  in  their  course,  descending  atrophy  of  the  nerve 
fibres  occurs,  but  this,  as  pointed  out  by  Uhthoff,  may  be  much 
less  complete  than  would  be  expected  ;  ultimately  in  the  later 
stages  atrophy  of  the  nerve  cells  occurs  as  a  consequence  of 
the  destruction  of  their  axis  cylinders. 

SYRINGOMYELIA. 

The  ocular  changes  which  occur  in  this  disease  may  be  con- 
sidered under  three  heads  :— 

(1)  Involvement  of  the  external  ocular  muscles. 

(2)  Pupillary  changes. 

(3)  Fundus  changes. 

(1)  Schlesinger 1  in  examining  300  cases  of  this  disease, 
found  paresis  or  paralysis  of  some  or  other  ocular  muscle  in 
thirty-three  cases.  The  sixth  nerve,  usually  on  one  side  only, 
is  the  one  most  often  affected,  but  the  third  nerve  may  be 
partly  involved.  When  these  nerves  are  affected,  it  probably 
implies  that  the  gliomatous  process  has  extended  as  high  as 
their  nuclei,  and  consequently  symptoms  of  involvement  of 
other  bulbar  nuclei  are  generally  present.2 

Spiller  has  reported  a  case  in  which  the  process  extended  as 
high  as  one  cerebral  peduncle. 

1  "  Die  Syringomyelie,"  Leipzig  u.  Wien,  1902. 

2  Starr,  Allbutt's  "  System  of  Med.,"  1910,  vii.,  862. 


MYASTHENLA   GRA  VIS  129 

The  paralysis  may  be  transient,  and  when  ptosis  occurs,  some 
care  is  needed  to  decide  whether  this  is  due  to  involvement  of 
the  oculo -motor  nerve  or  to  sympathetic  involvement. 

Nystagmus  is  a  fairly  common  symptom,  and  comes  on  early 
in  the  disease.  Schlesinger  found  fourteen  cases  of  true 
nystagmus,  and  thirty-seven  of  nystagmoid  movements,  among 
330  cases  of  syringomyelia  :  the  cause  of  the  nystagmus  is 
not  known. 

(2)  Pupillary  Changes. — These  changes  are  caused  by  involve- 
ment of  the  sympathetic  fibres  as  they  traverse  the  spinal  cord. 

Farquhar  Buzzard  J  says  :  "  Owing  to  the  frequent  involve- 
ment of  the  first  dorsal  and  eighth  cervical  segment  by  the 
gliomatous  process,  oculo -pupillary  symptoms  occur  in  about 
85  per  cent,  of  cases  ;  as  a  rule  the  paralysis  of  the  sympathetic 
fibres  is  unilateral,  or  is  more  advanced  on  one  side." 

If  the  fibres  of  the  sympathetic  are  involved  the  ordinary 
signs  will  be  present  (see  p.  96). 

(3)  Ophthalmoscopic    Changes. — These    are   quite    unusual, 
and  it  would  seem  clear  that  in  some  of  the  reported  cases  the 
change  is  due  to  other  conditions.     In  a  few  cases,  however, 
papillcedema  and  optic  atrophy  have  been  seen.     It  is  suggested 
that  the  former  is  due  to  internal  hydrocephalus  ;   cases  are 
reported  by  Saxer,2  Bullard  and  Thomas,3  and  Weisburg. 

MYASTHENIA   GRAVIS. 

Myasthenia  gravis  is  a  disease  which  is  characterised  by  a 
weakness  of  certain  of  the  voluntary  muscles  of  the  body  which 
is  slow  in  developing,  and  has  as  a  prominent  feature,  a  marked 
tendency  to  fluctuate  in  severity  from  day  to  day  or  from  week 
to  week,  or  even  to  disappear  for  months,  only  to  appear  again. 

There  are  no  sensory  symptoms.  The  sexes  are  equally 
affected,  the  average  age  of  onset  for  men  is  thirty-five,  for 
women  twenty -four.4 

Owing  to  the  rapid  tiring  of  muscles  there  is  difficulty  with 

1  Osiers  "  System  of  Med.,"  1915,  v.,  224. 

2  Beitrdg.  z.  *path.  u.  z.  allgem.,  xx.,  No.  11. 

3  Amer.  J.  of  Med.  Sci.,  1899,  cxvii.,  265. 

4  Campbell  and  Bramwell,  Brain,  1900,  xxiii.,  279. 


130  MYOTONIA   ATROPHICA 

speech,  mastication  and  swallowing.  The  levator  palpebrse  and 
other  external  muscles  of  the  eye  are  often  early  involved,  the 
intrinsic  muscles  escaping. 

Thus  one  of  the  earliest  signs  of  the  disease  may  be  ptosis  on 
one  or  both  sides,  diplopia  from  involvement  of  the  recti  or 
oblique  muscles,  or  lagophthalmos  from  weakness  of  the 
orbicularis.  The  ptosis  or  diplopia  may  be  marked  in  the 
morning,  much  less  by  mid-day,  and  conspicuous  again  by  night. 
The  muscles  show  the  myasthenic  electrical  reaction,  i.e., 
they  are  rapidly  exhausted  by  the  faradic  form  of  stimulation, 
but  are  little  affected  by  the  galvanic. 

The  condition  may  progress  slowly  for  many  years,  the 
manifestations  which  at  first  were  partial  and  temporary, 
becoming  complete  and  permanent. 

MYOTONIA   ATROPHICA. 

Myotonia  atrophica  is  a  familial  and  hereditary  disease,  the 
characteristic  features  of  which  are  weakness  of  the  facial 
muscles,  atrophy  of  the  vasti  of  the  thighs  and  dorsiflexors  of 
the  feet,  and  a  slow  relaxation  of  certain  muscles  after  con- 
traction (Batten  and  Gibb).1  In  addition,  the  subjects  of  this 
disease  are  especially  prone  to  the  early  development  of  cataract,  - 
a  point  which  distinguishes  it  from  any  other  form  of  myopathy. 
Thus  Greenfield  2  reports  thirteen  cases,  of  whom  eight  had 
cataract.  Fearnsides  3  reports  the  case  of  a  woman  who  had 
been  operated  on  for  double  cataract  at  the  age  of  thirty-one, 
and  of  whose  family  six  others  had  developed  cataract  before  the 
age  of  forty.  Three  of  these  showed  no  evidence  of  myopathy. 

The  cataracts  are  in  no  way  distinctive  except  in  the  early 
age — between  twenty  and  forty — at  which  they  are  developed, 
and  it  seems  from  Fearnside's  cases  that  for  a  long  time  they 
may  remain  the  only  manifestation  of  the  disease. 

1  Brain,  1909,  xxxii.,  187. 

a  Rev.  of.  Neural,  and  Psych.,  1911,  ix.,  170. 

3  Rev.  of  Neural,  and  Psych.,  1915,  xiii.,  312. 


£  —  $ 


CHAPTER   IV 

Renal     disease  —  Gout  —  Diabetes  —  Graves'     disease  —  Disease     of     the 
pituitary  body  —  Infantile  scurvy  —  Keratomalacia. 

RENAL   RETINITIS. 

THIS  term  is  used  to  embrace  those  changes  in  the  retina 
which  indicate  some  disease  of  the  kidney  ;  it  seems  to  be  more 
appropriate  than  the  term  albuminuric  retinitis,  since  the 
causes  of  albuminuria  are  so  diverse  and  are  not  necessarily 
located  in  the  kidney. 

The  ophthalmoscopic  appearances  are  very  characteristic,  but 
are  not  entirely  pathognomonic,  for  there  a  number  of  condi- 
tions, some  of  them  confined  to  the  eye,  which  closely  simulate 
the  retinitis  of  kidney  disease. 

Statistical.  —  The  General  Incidence  of  Retinitis.  —  The  inci- 
dence of  retinitis  in  kidney  disease  will  depend  very  largely 
upon  the  stage  at  which  the  examination  is  made,  and  this 
accounts  for  discrepancies  amongst  different  authors. 

It  is  clear  that  if  the  retina  is  examined  at  the  earliest  stage 
of  nephritis,  the  incidence  of  retinitis  will  be  nil,  whereas,  if  in 
a  series  of  cases  the  examination  be  made  immediately  before 
death,  the  incidence  is  very  high. 

Most  of  the  figures  which  are  given  by  authors  are  compiled 
from  patients  in  whom  the  disease  of  the  kidney  had  attained 
such  a  degree  as  to  render  treatment  within  the  wards  of  a 
hospital  advisable,  i.e.,  they  have  been  at  least  somewhat 
advanced  cases.  On  these  lines  I  have  notes  of  102  cases  which 
I  have  examined  ;  many  of  them  on  several  occasions  ;  from 
this  number  are  excluded  acute  nephritis,  trench  nephritis, 
pregnancy  cases,  arterio  -sclerotic  cases,  and  cases  in  which 
detachment  of  the  retina  has  developed. 

Of  these  102  cases,  thirty  -four  had  retinitis  and  sixty-eight 
had  it  not,  i.e.,  it  was  present  in  one-third  of  those  cases  of 


132  RENAL  RET  I  NIT  1 8 

nephritis  in  which  the  disease  had  attained  a  degree  sufficiently 
advanced,  to  render  in-patient  treatment  imperative  or 
advisable. 

Miles  Miley's  figures  arrived  at  in  a  similar  manner  l  are  in 
close  agreement  with  these  ;  he  found  fifty-two  cases  amongst 
166  patients  with  nephritis.  Elschnig,2  however,  found 
forty- three  cases  only  amongst  199  patients.  Herringham  3 
in  patients  under  forty  years  of  age,  found  twenty -six  cases 
of  retinitis  amongst  forty-six  patients. 

Incidence  in  the  Different  Forms  of  Nephritis. — A  primary 
difficulty  arises  from  the  fact,  that  as  pointed  out  by 
Herringham,  it  is  often  not  possible,  either  clinically  or  histolo- 
gically,  to  draw  a  sharp  line  of  distinction  between  the  different 
types  of  nephritis  ;  at  the  same  time  certain  broad  statements 
can  be  affirmed. 

Acute  Nephritis. — It  is  generally  agreed  that  if  pregnancy 
cases  are  excluded,  the  presence  of  retinitis  implies  a  nephritis 
of  considerable  standing  ;  it  is  believed,  however,  by  some, 
that  it  may  arise  occasionally  in  a  primary  acute  nephritis  ; 
thus  West 4  maintains  that  it  is  not  rare  in  this  condition. 
Sir  Wilmot  Herringham  states  :  "  Retinitis  albuminurica  has 
been  said  to  occur  in  acute  cases,  but  the  reports  are  uncon- 
vincing. As  a  practical  rule  its  existence  is  a  proof  that  the 
disease  is  of  long  standing." 

In  general  my  own  experience  is  in  accord  with  Herringham 's 
views  if  care  be  taken  to  exclude  those  cases  in  which  an  acute 
exacerbation  of  the  chronic  disease  simulates  a  primary  acute 
nephritis.  I  have  notes  of  thirty -four  cases  only  of  primary 
acute  nephritis,  but  I  have  examined  many  more  than  this,  and 
the  most  I  have  been  able  to  discover  has  been  a  small  haemor- 
rhage in  one  case,  and  in  three  the  disc  had  an  indefinite  hazy 
appearance  such  as  one  is  in  the  habit  of  attributing  to  oedema, 
but  which,  at  the  same  time,  it  is  difficult  to  be  sure  is  beyond 
physiological  limits. 

In  the  early  days  of  the  war  I  examined  the  fundi  of  119 

1  Trans.  Ophth.  Soc.  U.K.,  viii.,  1888,  134. 

2  Wiener  med.  Wochens,  1904,  494. 

"  Kidney  Diseases,"  London,  1912,  164. 
4  Lettsomian  Lectures,  1900  ;   Lancet,  February,  1916,  i.,  372. 


IN  DIFFERENT   FORMS  OF  NEPHRITIS      133 

consecutive  cases  of  soldiers  suffering  from  "  trench  nephritis," 
i.e.,  a  primary  acute  nephritis.1  Of  these  men  five  either  had 
retinitis  or  developed  it  whilst  they  were  under  observation, 
and  in  only  one  of  them  was  there  any  discoverable  suspicion 
that  he  was  previously  the  subject  of  chronic  nephritis.  The 
interval  between  the  onset  of  the  symptoms  of  nephritis  and 
of  the  retinitis  varied  from  seven  to  thirteen  weeks,  in  one  case 
the  date  of  the  onset  of  symptoms  was  precisely  known,  and  the 
actual  onset  of  the  retinitis  was  seen  to  commence  nine  weeks 
later,  whilst  he  was  in  hospital. 

Whether  these  cases  are  considered  to  have  persisted  long 
enough  to  be  called  chronic  nephritis  as  they  probably  became, 
or  whether  they  should  rather  be  labelled  acute,  I  do  not  know  ; 
but  if  the  latter  view  is  taken,  it  is  clear  that  the  question  as  to 
whether  retinitis  occurs  in  acute  nephritis  must  be  answered  in 
the  affirmative  as  regards  this  particular  variety  of  the  disease, 
and  probably  also  in  pregnancy  cases. 

Chronic  Parenchymatous  Nephritis. — There  is  a  consensus  of 
opinion  that  retinitis  is  not 'common  in  this  form  of  the  disease. 
Of   fifty   cases   in   patients   under   the   age   of   twenty-one 
examined  by  Nettleship  2  seven  only  had  retinitis. 

Of  twenty-three  fatal  cases  of  my  own  in  which  a  definite 
differential  diagnosis  of  chronic  parenchymatous  nephritis  was 
made,  seven  had  retinitis. 

Chronic  Interstitial  Nephritis. — In  this  form  of  nephritis 
retinitis  is  frequent,  if  the  period  of  examination  extends  to  the 
end  of  the  patient's  life. 

Of  my  own  cases  there  were  forty -four  in  which  an  unqualified 
diagnosis  of  chronic  interstitial  nephritis  was  made  by  the 
physician,  and  of  these  exactly  one-half  had  some  degree  of 
retinitis,  and  four  besides  had  retinal  haemorrhages  only.  Of 
eighty  cases  under  the  age  of  twenty-one  examined  by 
Nettleship  2  thirty-one  had  either  retinitis  or  multiple  haemor- 
rhages. 

I  am  convinced  that  very  few  patients  die  in  uraemia  without 
changes  in  the  eye  grounds.  Thus  I  have  collected  twenty 

1  Lancet,  1915,  ii.,  1348. 

2  Royal  London  Ophth.  Hosp.  Reports,  1906,  xvi.,  3. 


134  RENAL   RET  I  NIT  IS 

cases  which  I  had  examined  ophthalmoscopically  at  St.  Bartho- 
lomew's Hospital,  who  afterwards  died  with  a  clinical  diagnosis 
of  uraemia,  and  in  whom  post-mortem  findings  were  in  accord 
with  the  clinical  diagnosis  in  every  case  ;  in  eight  of  them  a 
detailed  report  of  the  microscopic  appearances  of  the  kidneys 
was  available.  Twelve  were  males  and  eight  females,  the 
average  age  of  the  males  was  thirty -seven,  that  of  the  females 
was  forty -three,  the  average  weight  of  the  hearts  was  18  ounces, 
the  average  systolic  blood  pressure  was  202*5  mm.,  and  the 
average  weight  of  the  kidneys  together  was  8  ounces.  Of  these 
twenty  cases  nineteen  had  retinitis,  and  the  one  remaining  was 
last  examined  three  weeks  before  death. 

Sex  Incidence. — The  sexes  are  equally  liable  to  retinal 
disease,  but  since  chronic  nephritis  is  about  twice  as  common  in 
adult  men  as  in  adult  women  so  also  is  retinitis  about  twice  as 
frequent,  as  is  shown  by  the  following  figures  :— 

Males.  Females. 

Nettleship      .          .          .          .56  .  .          30 l 

Miles  Miley    .          .          .          .38  ..          13  2 

Bull 56  .  .          47  3 

Foster  Moore           .          .          .63  .  .          39 

In  younger  patients,  however,  the  sex  incidence  of  nephritis 
is  reversed,  for  Nettleship  4  found  that  of  fifty-one  cases  of 
chronic  interstitial  nephritis  in  children  under  the  age  of 
thirteen,  30  per  cent,  were  males  and  70  per  cent,  females,  and 
correspondingly  retinitis  becomes  more  frequent  absolutely  in 
females. 

Age  Incidence. — The  youngest  in  whom  I  have  found  a 
record  of  renal  retinitis  is  in  a  female  chilcT  of  five  reported  by 
Bull. 

Nettleship    records    two    aged    seven,    three    aged    eight ; 
Herringham  one  aged  seven. 
/  I  have  seen  it  in  a  child  of  seven  whose  blood  pressure  was 

1  Royal  London  Ophth.  Hosp.  Reports,  1903,  xv.,  327. 

2  Trans.  Oph.  Soc.  U.K.,  1888,  viii.,  134. 
8  Trans.  Amer.  Op.  Soc.,  1886,  iv.,  185. 

*  Royal  London  Ophth.  Hosp.  Reports,  1906,  xvi.,  5. 


135 
dX* 

260  mm.,  and  in  another  child  of  the  same  age  with  a  blood 
pressure  of  240. 

Of  my  cases  the  average  age  of  those  with  retinitis  was  37-5, 
and  of  those  without  retinitis  31  ;  64  per  cent,  of  Nettleship's 
and  88  per  cent,  of  Miles  Miley's  patients  were  between  the 
ages  of  thirty  and  sixty. 

Prognostic  Significance. — There  is  no  doubt,  that  if  arterio- 
sclerotic,  pregnancy,  and  trench  nephritis  cases  be  excluded,  the 
long-accepted  formula,  that  patients  seldom  live  so  long  as  two 
years  after  the  discovery  of  retinitis,  is  true. 

The  prognosis  is  slightly  less  serious  in  private  than  in  hospital 
patients. 

Belt  l  found  that  of  419  patients,  6  per  cent,  lived  longer  than 
two  years,  and  that  65  per  cent,  died  within  a  year.  Miley 
found  in  forty-five  cases  that  the  average  duration  of  life  from 
the  time  of  discovery  of  the  retinitis  was  four  months.  Bull  2 
found  that  of  103  patients,  eighty-six  were  dead  ;  and  of  these 
eighty-six,  seventy-five  died  within  two  years,  four  were  alive 
four  years  later,  one  six  years,  and  one  seven  years  later.  And 
of  twenty -four  patients  of  Nettleship's  under  the  age  of  twenty- 
one,  one  only  lived  longer  than  two  years. 

The  prognosis  is  no  less  grave  in  children  than  in  adults ; 
indeed  it  is  stated  that  in  elderly  patients  the  presence  of 
retinitis  implies  a  less  grave  prognosis  than  in  young  patients. 
This  statement,  however,  is  probably  based  on  the  inclusion 
of  cases  of  arterio -sclerotic  retinitis  with  the  true  renal  cases. 

The  "man  from  whom  Figs.  50  and  51  (pp.  151  and  153)  were 
made  was  first  seen  by  me  in  May,  1913,  with  severe  renal  retinitis, 
a  detachment  of  each  retina,  and  a  blood  pressure  of  215  mm. 
In  1915  he  was  exhibited  at  the  Congress  of  the  Ophthalmo- 
logical  Society  of  the  United  Kingdom  3  as  showing  consider- 
able subsidence  of  retinitis  and  complete  disappearance  of  the 
retinal  detachments.  He  worked  hard  in  a  small  arms  factory 
during  the  war,  and  in  June,  1920,  was  still  in  full  work,  and 
expressed  himself  as  feeling  very  well.  He  had  marked 

1  Amer.  Med.  Ass.,  1895,  xxv.,  735. 

2  Trans.  Amer.  Ophth.  Soc.,  1886,  iv.,  190. 

3  Trans.  Ophth.  Soc.  U.K.,  1915,  xxxv.,  159. 


136  RENAL  RETINITIS 

secondary  retinal  and  nerve  atrophy,  albuminuria,  and  a 
systolic  blood  pressure  of  260  mm.  He  died  in  hospital  in 
August,  1920,  in  urssmia  without  a  recurrence  of  retinitis  (vide 
infra).  Thus  he  lived  in  what  he  called  good  health,  for  seven 
years  and  three  months  after  the  development  of  so  severe  a 
retinitis  as  to  cause  retinal  detachment  in  each  eye. 

The  Relation  of  Blood  Pressure'  to  the  Development  of 
Retinitis. — As  the  renal  disease  progresses  in  severity,  it  is  to 
be  expected  that  so  also  will  the  concomitant  signs  and  lesions 
increase,  so  that  the  more  advanced  the  disease,  the  higher  in 
general  will  be  the  blood  pressure,  and  the  greater  the  incidence 
of  retinitis. 

In  sixty-eight  of  my  cases  of  nephritis  the  systolic  blood 
pressure  is  recorded,  thus  : — 

Of  twenty-three  patients  with  retinitis  the  average 
systolic  blood  pressure  was  211  mm.,  and  in  six  cases  only 
was  it  less  than  200  ; 

Whilst  of  forty-five  patients  without  retinitis  the  average 
systolic  blood  pressure  was  150,  and  in  nine  only  did  it 
exceed  160  mm. 

And,  again,  of  seventy-five  soldiers  with  trench  nephritis  the 
average  blood  pressure  of  seventy  with  normal  fundi  was 
143  mm.,  whilst  in  five  in  whom  retinitis  developed,  it  was 
180  mm. 

Thus  it  is  very  unusual  to  meet  with  retinitis  except  in  the 
presence  of  a  considerably  increased  blood  pressure,  but 
retinitis  is  often  absent  though  the  blood  pressure  is  greatly 
raised. 

The  Retinitis  of  Pregnancy. — It  is  freely  recognised  that 
when  retinitis  occurs  in  association  with  the  nephritis  of  preg- 
nancy, the  patient  not  being  the  subject  of  an  antecedent 
chronic  nephritis,  it  conveys  a  much  less  grave  prognosis  than 
in  chronic  nephritis  in  general. 

Not  only  may  the  patient  live  for  many  years,  but  the  retinitis 
may  become  completely  resolved  and  leave  very  little  ophthal- 
moscopic  residue,  and  the  sight  is  often  in  large  measure 
restored. 


RET  I  NIT  1 8  IN  PREGNANCY 


137 


Statistical. — Nettleship  *  followed  up  twenty-two  cases  of  the 
condition  for  many  years,  and  of  them  one  only  was  known  to 
have  died  within  two  years  ;  four  were  known  to  have  died 
three-and-a-quarter,  four-and-a-half,  seven,  and  seven,  years 
later,  and  six  others  were  known  to  be  alive  for  periods  ranging 
up  to  nine  years. 

Retinitis  does  not  as  a  rule  recur  in  future  pregnancies  where 
it  has  once  been  present  and  subsided. 

It  most  often  develops  after  several  pregnancies  though  it 
may  occur  at  the  first ;  thus 
amongst   nineteen    cases    it 
occurred  at  or  after  the  fifth 
pregnancy  in  fourteen. 

In  three  of  the  cases  which 
occurred  during  later  preg- 
nancies, the  patients  were 
seen  after  eleven,  thirteen, 
and  twenty -four  years,  and 
had  had  no  recurrence  of  eye 
symptoms. 

It  is  suggested  by  Semple  2 
that  retinitis  in  pregnancy 
is  not  directly  related  to  the 
kidney  disease,  but  that  it, 


FIG.  41. — To  show  a  large  globular  retinal 
detachment  in  a  case  of  renal  retinitis 
from  a  case  of  nephritis  in  pregnancy. 
A  similar  detachment  was  present  in 
the  other  eye. 


and  the  involvement  of  the 

kidney,  and  liver,  and  other 

organs,  are  due  to  a  common  toxaemia,  and  consequently  that 

one  or  other  of  these  organs,  the  eye  for  instance,  may  be 

involved,  the  other  tissues  escaping. 

Thus  Posey  and  Hirst3  report  a  case  with  constitutional 
symptoms,  with  normal  urine  but  with  very  extensive  retinitis  ; 
an  early  abortion  procured  complete  recovery  of  vision  ;  Semple 
reports  a  similar  case. 

In  summing  up  the  characteristics  of  the  retinitis  of  preg- 
nancy, Semple  remarks  that  it  differs  from  other  forms  of  renal 


1  Royal  Land.  Ophth.  Hosp.  (Moorfields)  Reports,  1903,  xv.,  322. 

2  Trans.  Amer.  Ophth.  Soc.,  1909-11,  xii.,  817. 

3  Jour.  Amer.  Med.  Ass.,  1908,  1.,  865. 


138  RENAL  RET  IN  IT  IS 

retinitis  ;  he  says  :  "  The  sudden  onset,  the  tremendous  exuda- 
tion into  the  retina,  the  equally  sudden  cessation  on  removal 
of  the  cause  if  done  promptly  enough,  the  subsequent  and  com- 
plete restitution  of  visual  acuity,  all  are  in  marked  contrast  to 
the  usual  course  of  retinitis  of  other  forms  of  Bright's  disease." 

The  retinal  changes  in  these  pregnancy  cases  are  usually 
very  severe,  with  copious  exudate  and  oedema  in  the  retinal 
tissues,  and  detachment  of  the  retina  occurs  from  the  pouring 
out  of  serum  into  the  subretinal  space,  in  a  much  larger  pro- 
portion of  them  than  in  other  forms  of  nephritis  (Fig.  41). 

Thus,  of  forty -four  cases  of  detachment  of  the  retina  in  renal 
retinitis  l  which  I  was  able  to  collect,  thirty  occurred  in  women, 
and  of  these  seventeen  were  associated  with  pregnancy.  And 
whereas  ten  of  these  seventeen  are  known  to  have  recovered, 
temporarily,  of  the  thirteen  unassociated  with  pregnancy  eleven 
are  known  to  have  died,  and  two  only  recovered  for  the  time 
being. 

In  addition  to  the  loss  of  sight  which  results  from  the  visible 
morbid  changes  in  the  retina  which  are  included  under  the 
term  of  the  retinitis  of  pregnancy,  marked  visual  defects  may 
occur  during  pregnancy,  parturition,  or  the  puerperium  without 
discoverable  retinal  disease,  and  whilst  they  are  often  asso- 
ciated with  signs  of  eclampsia,  they  are  not  necessarily  so 
connected. 

Adam  2  found  visual  disturbances  in  50  per  cent,  of  cases  of 
eclampsia. 

The  loss  of  sight  which  is  rapid  and  complete,  may  last  from 
a  few  hours  up  to  several  weeks,  but  recovery  is  complete. 

The  fundi,  except  for  an  occasional  flame-shaped  haemorrhage, 
are  normal,  and  the  pupils  remain  active  to  light  stimulus. 

These  cases  have  been  examined  by  Stephenson  and  Her- 
ringham,3  who  show  that  they  are  not  dependent  upon  ursemia, 
although  they  have  often  been  confused  with  it. 

They  are  usually  associated  with  scanty  urine  which  contains 
albumen,  blood,  and  casts  ;  with  oedema,  and  headaches. 

1  Foster  Moore,  Royal  Land.  Ophth.  Hosp.  Reports,  1916,  xx.,293. 
4  Monats.f.  Geburts.  u.  Gynak..  1914,  xxxix.,  808. 
8  "Ophthalmoscope,"  1910,  viii.,  168. 


OPHTHALMOSCOPIC  CHANGES  139 

It  has  been  suggested  1  that  the  immediate  cause  is  an 
exhaustion  of  the  cerebral  visual  centres,  which  similarly, 
when  other  parts  of  the  brain  cortex  are  affected,  results  in 
transient  aphasia,  hemiparesis,  etc. 

In  some  cases  of  nephritis,  especially  where  uraemia  is  present 
or  imminent,  exophthalmos  occurs.  I  have  seen  a  case  in 
which  it  was  well  marked  and  associated  with  pain  behind  the 
eyeballs  and  limitation  of  movement  of  the  eyes  ;  the  fundi  at 
first  were  normal,  but  eleven  weeks  later,  conspicuous  pigmen- 
tary proliferation  was  seen  with  the  ophthalmoscope,  marking 
out  the  course  of  the  choroidal  vessels,  it  seemed  as  if  toxins 
in  the  orbital  tissues  had  produced  oedema  of  them  and  pain 
and  limitation  of  movement  of  the  eyes,  and  had  involved  the 
posterior  ciliary  arteries  so  as  to  cause  marked  proliferation  of 
pigment  in  their  course. 

The  occurrence  ^of  exophthalmos  and  of  some  other  ocular 
signs  in  chronic  nephritis  has  been  especially  studied  by  Barker 
and  Hanes,2  who  insist  upon  their  frequency.  They  state 
that  of  thirty -three  consecutive  cases  seen  in  the  Johns  Hopkins 
Hospital,  exophthalmos  was  present  in  48-4  per  cent.,  and 
that  of  seven  consecutive  fatal  cases  exophthalmos  was  an 
obvious  sign  in  all.  They  say,  in  addition,  that  Graefe's  sign, 
Stellwag's  sign,  Moebius'  sign,  and  inequality  of  the  pupils  are 
all  frequently  seen. 

Ophthalmoscopic  Changes  in  General. — These  are  divisible 
into  three  kinds,  exudates,  haemorrhages,  and  oedema,  and  in 
cases  of  any  marked  extent  all  of  these  will  be  present. 

There  is  no  constancy  in  the  order  in  which  they  appear. 
Sometimes  the  first  sign  is  a  retinal  haemorrhage,  at  other  times 
swelling  of  the  nerve  head,  or  the  presence  of  one  or  other 
variety  of  retinal  exudate. 

Some  degree  of  oedema  of  the  retina  is  probably  present  in 
all  cases,  but  the  ophthalmoscopic  evidences  of  it  are  very 
indefinite,  and  unless  the  oedema  is  well  marked,  it  is  not  pos- 
sible to  diagnose  its  presence  or  absence  with  any  certainty  by 
means  of  the  ophthalmoscope,  and  were  it  not  for  histological 

1  Garrod  and  Thursfield,  "  Children's  Diseases,"  1913,  729. 

2  Amer.  Jour,  of  Med.  Set.,  1909,  cxxxviii.,  669. 


140  RENAL  RETINITJS 

evidence,  and  one's  general  knowledge  of  such  pathological 
processes,  one  would  be  left  in  doubt  as  to  its  occurrence  except 
in  a  few  cases. 

The  microscope,  however,  demonstrates  how  marked  the 
oedema  may  be,  and  in  .some  cases  it  may  transgress  the 
limiting  membranes  of  the  retina  and  produce  a  detachment  of 
this  membrane  by  collecting  in  the  subretinal  space  (Fig.  41  and 
p.  150). 

Haemorrhages. — Retinal  haemorrhages  are  always  present, 
and  are  most  frequent  and  largest  in  the  central  regions. 

They  are  seldom"  very  large,  are  usually,  though  by  no  means 
always,  of  the  flame-shaped  type,  and  undergo  changes  similar 
to  like  haemorrhages  from  other  causes  (vide  p.  34). 

So  long  as  active  changes  are  present  haemorrhages  will  be 
found,  their  presence  being  maintained  by  the  continual 
occurrence  of  fresh  ones  as  the  older  ones  become  absorbed, 
but  as  a  stage  of  complete  subsidence  is  reached  if  the  patient 
survives  sufficiently  long,  so  the  haemorrhages  completely 
disappear. 

Exudates. — Any  local  degenerative  process  in  the  retina  will 
impair  its  local  transparency,  and  so  will  appear  to  the  ophthal- 
moscope as  a  white  spot  or  patch,  and  similarly  areas  of  exudate 
amongst  the  retinal  tissues  are  seen  as  white  spots  or  areas ; 
indeed,  pathological  processes  of  most  diverse  origin,  will 
produce  spots  or  areas  in  the  retina  which  are  white  in  colour ; 
they  differ,  however,  as  regards  their  size,  texture,  arrangement 
and  progress. 

A  number  of  white  areas  of  differing  appearance  occur  in 
the  retina  in  renal  retinitis  and  can  be  seen  to  undergo  various 
metamorphoses  under  observation. 

(1)  Cotton  Wool  Patches. — Under  this  descriptive  head  are 
included  certain  soft-edged,  woolly-looking  patches  of  con- 
siderable size,  which  occur  around  the  central  regions  of  the 
retina.  They  are  often  partly  fringed  or  overlain  by  blood,  and 
may  obscure  the  retinal  vessels  ;  evidence  of  retinal  oedema 
may  be  seen  around  them. 

They  occur  especially  in  those  cases  in  which  the  toxic 
element  of  nephritis  is  prominent.  They  are  often  copious. 


141 


and  arc  seen  in  their  characteristic  form  in  pregnancy  cases 
and  in  parenchymatous  nephritis,  but  occur  in  other  forms  of 
the  disease.  It  is  in  the  presence  of  these  patches  that  oadema 
is  most  marked,  and  that,  therefore,  retinal  detachment  is 
most  likely  to  occur.  The  separate  patches  may,  by  confluence, 
give  rise  to  large  areas  of  retinal  infiltration. 

Histologically,  this  exudate  is  represented  by  an  extensive 

B 


—A 


I'm.  42. — Section  through  the  retina  in  a  case  of  severe j-etinitis  in  chronic 
parenchymatous  nephritis.  All  the  layers  of  the  retina  are  profusely 
infiltrated  by  oedema  and  fibrinous  exudate.  A.  Layer  of  rods 
and  cones.  B.  Nerve  fibre  layer. 

infiltration  of  the  retinal  layers  by  a  fine  fibrinous  mesh  work, 
which  stains  well  with  Weigert's  anilin-gentian-violet  stain 
(Fig.  42).  It  often  occurs  in  flask-like  spaces  in  Henle's  layer, 
but  may  infiltrate  all  the  retinal  tissues. 

Three  stages  are  described  by  Semple  1  in  the  metamorphosis 
of  this  exudate,  a  view  which  is  supported  by  Rochon 
Duvigneaud.2 

1  Trans.  Amer.  Ophth.  Soc.,  1909-11,  xii.,  817. 
-  Soc.  frany.  tFOphlhalntol.,  1912,  xxix.,  (5. 


142 


RENAL  RETINITIS 


(}\  Network  of  fibrin  (Fig.  42). 

(2)  The  masses  lose  their  fine  fibrillar  appearance  and  cease 

to  stain  by  Weigert's  stain. 

(3)  All  fibrillar  appearance  is  lost  and  the  exudate  takes  on 

the  form  of  masses  of  hyalin  material  (Fig.  43). 


FIG.  43. — A  section  through  the  retina  near  the  yellow  spot  in  a  case  of 
renal  retinitis  in  which  a  star  figure  was  present.  There  has  been  a 
large  amount  of  hyaline  exudate  in  Henle's  layer  which  has  been 
very  nearly  removed  by  the  large  globular  phagocytic  cells  ;  a  little 
still  remains  between  these  cells.  The  rods  and  cones  appear  to  have 
completely  degenerated.  A.  Rod  and  cone  layer.  B.  Henle's  layer 
with  exudate  and  phagocytic  cells. 

After  a  time  conspicuous  large  globular  phagocytic  cells 
appear  and  apply  themselves  to  the  hyalin  masses  and  bring 
about  their  absorption  (Fig.  43).  The  cells  themselves  become 
filled  with  a  lipoid  material,  and  some  of  them  are  densely 
stained  by  osmic  acid  (Figs.  44  and  45).  It  is  evident  that  fat 
is  formed  in  the  retina  in  some  cases  at  least.  Semple  could 


RETINAL  EXU DATES 


find  no  evidence  of  fat  in  his  cases.  He,  however,  emphasises  the 
fact  that  they  were  early  cases.  No  fat  was  found  by  Litten.1 
On  the  other  hand,  fat  was  found  by  Leber,2  Lauber  and 
Adamiik,3  Rochon  Duvigneaud  and  Ma  was,4  and  Ginsberg.5 

The  individual  "  cotton  wool  "  patches  can  be  seen  with  the 
ophthalmoscope  to  disappear  in  course  of  time,  although  at  the 


— B 


. 


FIG.  44. — A  section  through  the  retina  of  a  young  woman  who  died  of 
chronic  interstitial  nephritis  and  in  whom  a  well-marked  star  figure 
was  present  in  the  retina.  Many  large  phagocytic  cells  are  present, 
which  are  full  of  fat  stained  by  osmic  acid ;  the  cells  are  almost 
entirely  confined  to  Henle's  layer,  three  only  transgressing  its  limits, 
the  greater  number  are  in  rather  close  apposition  with  the  external 
limiting  membrane.  A  marks  the  nerve  fibre  layer,  and  B  the  rod 
and  cone  layer. 

same  time  fresh  areas  may  be  developing.  As  a  rule  they  simply 
fade  away  and  leave  no  trace  behind.  The  larger  the  mass  the 
longer  does  it  take  to  become  absorbed.  I  have  seen  a  small 
patch  completely  disappear  in  fourteen  days  ;  I  have  also  seen 

1  Milnch.  Med.  Wochensch.,  1903, 1.,  391. 

2  v.  Graefe's  Archiv.  f.  Ophth.,  1909,  Ixx.,  20. 

3  v.  Graefe's  Archiv.  f.  Ophth.,  1909,  Ixxi.,  429. 

4  Mem.  et  Bull,  de  la  Soc.  Franc.  (FOphth..  1912,  xxix.,  6. 

5  v.  Graefe's  Archiv.  fur  Ophth.,  1912,  Ixxxii.,  1. 


144 


RENAL  RETLNITIS 


a  larger  solid-looking  area  remain  comparatively  little  changed 
after  seven  months  (Fig.  53,  p.  157). 

The  Star  Figure. — The  most  striking  form  of  exudate  is  that 
which  occurs  in  lines  radiating  from  the  region  of  the  yellow 
spot  giving  rise  to  the  familiar  star  figure. 

Not  infrequently  the  first  sign  of  retinitis  is  in  the  form  of 

-A 

ii 

•  — B 


Fiu.  45. — From  the  same  patient  as  Fig.  44  more  highly  magnified.  A 
well-developed  star  figure  was  present.  Notice  the  distribution  of 
the  large  fat-containing  phagocytic  cells.  A  marks  the  nerve  fibre 
layer,  B  the  layer  of  ganglion  cells,  and  C  the  rod  and  cone  layer. 

one  or  more  rod-like  or  oat-shaped  dots  along  a  line  of  radius 
from  the  yellow  spot.  These  spots  are  added  to  until  in  the 
course  of  some  weeks  the  fully-formed  star  is  seen  (Fig.  46, 
p.  145). 

In  some  cases,  previous  to  the  development  of  the  star,  a 
complete  figure  of  very  fine  lines  may  be  seen  radiating  all 
round  from  the  yellow  spot  as  a  centre. 


r.  *    o> 

o     c/^    r 


0 

0 


MRCH2 


*       v     »a     * 

-X  \^o,p    ^^ 
,^A  M/  ^  ' 


o 


A  PL.  24  MAY     10 

FIG.  46.- — Showing  the  slow  development  of  a  star  figure  in  a  girl  oi 
twenty-one  under  the  care  of.  Dr.  James  Calvert ;  she  had  had 
symptoms  of  nephritis  for  two  years.  Except  in  the  last  figure,  an 
endeavour  was  made  to  represent  every  individual  spot  of  exudate 
that  was  visible.  The  dates  are  those  at  which  each  drawing  was 
made.  The  small  circle  in  each  figure  represents  the  position  of  the 
yellow  spot. 

).  10 


146  RENAL   RETINITIS 

These  lines  have  the  appearance  of  refractile  lines,  or  lines  of 
tension,  and  suggest  a  sort  of  scaffolding  around  which  a  star 
figure  may  be  laid  down ;  they  extend  outwards  beyond  the 
limits  of  the  star  which  follows. 

I  have  watched  the  development  of  a  star  figure  from  the 
time  when  two  dots  only  were  present  till  a  well-developed 
figure  was  seen  in  the  course  of  twelve  weeks  (Fig.  46). 

I  have  seen  the  star  form  and  its  identity  afterwards  become 
lost  in  a  profusion  of  exudate  in  the  course  of  seven  weeks,  and 
more  than  once  I  have  watched  one  completely  disappear  in  the 
course  of  a  good  many  months — nine  in  one  case — and  leave  no 
trace  behind  of  its  former  presence. 

This  very  striking  formation  is  more  often  seen  in  renal 
retinitis  than  in  any  other  condition,  but  it  is  by  no  means 
pathognomonic  of  nephritis,  for  it  is  seen  in  a  number  of  very 
diverse  forms  of  retinal  disease,  e.g.,  papilloedema,  embolism  of 
the  retinal  artery,  syphilitic  retinitis,  thrombosis  of  the  retinal 
veins,  etc. 

Unless  the  actual  fovea  is  the  site  of  exudation,  the  vision  is 
not  necessarily  impaired. 

With  regard  to  the  explanation  of  the  formation  of  this 
striking  figure,  it  is  usually  attributed  to  the  arrangement  of  the 
fibres  in  Henle's  layer,  that  in  fact  it  has  an  anatomical  basis. 

Perhaps  one  may  be  permitted  to  put  forward  tentatively  a 
physical  cause  as  the  basis.  As  has  been  mentioned,  it  is  by 
no  means  rare  for  the  star  figure  to  be  preceded  by  closely 
packed  fine  lines  radiating  outwards  from  the  macula,  or  in  the 
presence  of  a  star  these  lines  may  be  seen  to  extend  well  beyond 
it  all  round,  and  as  has  been  stated,  they  suggest  a  scaffolding 
around  which  the  figure  is  laid  down.  These  lines  are  so  fine, 
so  strictly  linear,  and  so  regular  in  arrangement,  as  to  suggest 
most  strongly  lines  of  tension  in  an  cedematous  retina ;  they 
certainly  have  no  organic  basis.  It  suggests  itself,  that  were 
the  yellow  spot  held  down  to  the  underlying  pigment  layer,  the 
rest  of  the  retina  being  free,  on  the  development  of  retinal 
oedema  at  the  posterior  pole  of  the  eye,  these  lines  of  tension 
would  tend  to  form.  I  know  of  no  histological  evidence  that 
any  such  adhesion  is  present,  I  therefore  examined  thirty -seven 


THE  STAR  FIGURE 


147 


eyes  in  the  post-mortem  room.  In  thirty-three  of  these  I  could 
find  no  evidence  of  adhesion  at  this  spot,  in  four  it  seemed  to 
be  slightly  adherent,  and  it  was  common  to  find  with  the  sliding 
of  the  retina  on  the  underlying  pigment  layer,  that  the  retina 
became  puckered  at  the  macula  as  if  it  were  held  here. 

Some  support  for  this  suggestion  may  be  derived  from  the 


' 


FIG.  47. — A  section  in  a  plane  parallel  with  the  surface  of  the  retina 
passing  through  the  ganglion  cell  layer  in  the  central  part  and 
through  the  nerve  fibre  layer  around.  The  section  was  immediately 
.to  one  side  of  the  yellow  spot,  over  an  area  where  a  well -developed 
star  figure  was  present.  It  shows  the  entire  absence  of  exudate  in 
this  layer,  and  no  change  other  than,  perhaps,  some  degeneration  in 
some  of  the  ganglion  cells.  Sections  at  a  deeper  level  over  this  area 
showed  the  presence  of  copious  exudate. 

fact,  that  it  is  not  rare  for  a  star  arrangement  of  exudate  to 
develop  in  cases  of  papillcedema,  and  in  my  experience  it  is 
seldom  a  complete  star  that  is  formed,  but  a  fan-shaped  figure. 
In  this  latter  case  the  fan  is  always  placed  between  the  disc 
and  the  yellow  spot,  with  its  apex  towards  the  latter.  That 
is,  the  fan  figure  is  formed  in  that  portion  of  the  retina  to  which 
the  oedema  spreads  from  the  nerve  head,  and  is  laid  down  along 

10—2 


148  RENAL  RETINITIS 

supposed  lines  of  tension  which  run  between  two  fixed  points, 
the  disc  and  the  yellow  spot. 

Hulke  believed  the  star  was  due  to  fatty  transformation  of 
hyperalbuminous  fluid  and  fibrogenous  serum.  Nuel  attributes 
it  to  the  presence  of  globules  of  an  albuminoid  nature  in  Henle's 
layer,  and  Rochon  Duvigneaud  suggests  that  it  is  caused  by  the 
presence  of  fibrinous  clots  in  the  nerve  fibre  layer  and  to  the 
radial  disposition  of  the  nerve  fibres  around  the  macula.  (See, 
however,  Fig.  47.) 

It  is  probable  that  the  exudate  itself  is  of  a  hyaline  type  (Figs. 
43  and  45) ,  and  that  exudate  of  a  fibrinous  nature  is  not  kept 
within  such  bounds  as  to  form  a  star  figure  (Fig.  42,  p.  141). 

Other  white  patches  are  produced  by  degenerations  in  the  nerve 
fibre  layer  resulting  in  patches  of  varicose  nerve  fibres  and  cytoid 
bodies  (Fig.  48).  Ginsberg  looks  upon  these  as  one  of  the  chief 
sources  of  the  white  patches.  They  are  seen  with  the  ophthal- 
moscope as  white  areas  with  harder  edges  than  the  fibrinous 
patches,  they  are  slower  to  disappear,  and  may  show  cholesterin 
crystals  within  them.  They  occur  in  a  number  of  retinal  de- 
generations, and  are  by  no  means  limited  to  renal  retinitis. 

Semple  believes  that  degeneration  of  the  retinal  ganglion 
cells  is  responsible  for  some  of  the  white  areas. 

Vascular  Changes. — The  changes  in  the  veins  are  not  impor- 
tant, and  whilst  in  long-standing  cases  sclerosis  of  the  arteries 
occurs,  the  arterial  disease  is  believed  by  many  to  be  of  a  secon- 
dary nature. 

Thus  Semple,  Schieck,1  Opin  and  Rochon  Duvigneaud,2  and 
Lauber  and  Adamiik,  all  look  upon  the  changes  in  the  vessels 
as  of  secondary  importance  in  the  production  of  the  retinal 
changes,  the  changes  being  dependent  upon  toxins  in  the  blood. 

Semple  points  out  that  there  are  cases  of  marked  involve- 
ment of  the  retina  in  Bright's  disease  where  the  retinal  and 
choroidal  vessels  are  practically  normal.  He  believes  that  the 
retinal  disease  is  due  to^toxins  in  the  blood  acting  on  the 
retinal  tissues,  before  it  affects  the  vessels  to  an  extent  his- 
tologically  demonstrable. 

1  Ophthalmol.  Gesellsch.  Heidelberg,  1907,  77. 

2  J.  de  physiol.  el  de  path.,  1903,  v.,  1081. 


Papillcedema. — It  is  of  considerable  importance  to  have  in 
mind,  that  whilst  in  any  case  of  severe  retinitis  the  optic  disc  is 
involved,  yet  at  times  a  pure  papilloedema  may  occur  as  a 
manifestation  of  renal  disease.  This  papill oedema  may  be  quite 
indistinguishable  from  that  seen  with  a  cerebral  tumour,  and 
whilst  as  time  goes  on  exudates  usually  appear  in  the  surrounding 
retina  wide  of  the  disc  which  make  the  nature  of  the  case  clear, 
I  have  seen  two  cases  continue  up  to  their  death  with  a  pure 


— B 


FIG.  48. — Section  through  the  retina  in  a  case  of  renal  retinitis  to  show 
varicose  nerve  fibres  in  the  nerve  fibre  layer,  B.  A.  Rod  and  cone 
layer. 

papillosdema,  and  without  exudates  in  the  retina  around 
(Fig.  49). 

I  have  once' seen  a  well-marked  unilateral  papillcedema  in 
renal  disease.  The  patient,  a  woman,  died  in  uraemia,  a  fact 
which  was  confirmed  by  post-mortem  examination.  As  late  as 
two  days  before  her  death  the  left  disc  was  in  a  condition  of 
well-marked  papillcedema ;  it  was  indistinguishable  from  that 
seen  with  a  cerebral  tumour,  whilst  the  other  disc  was  quite 
normal  in  appearance. 

It  is  obvious,  seeing  that  headaches  and  vomiting  are  pro- 
minent features  of  both  renal  disease  and  of  a  cerebral  tumour, 


150 


RENAL  RETINITIS 


and  that  a  pure  papillcedema  may  occur  in  either,  that  a  real 
difficulty  may  arise  at  times  in  distinguishing  between  these 
two  conditions. 

A  further  complication  too  is  supplied  by  the  fact  that  in  a 
few  cases,  especially  in  young  subjects,  a  partial  star  figure  may 
develop  in  the  papillredema  associated  with  a  cerebral  tumour. 

My  impression  is  that  the  prognosis  is  especially  grave  in 
these  cases  ;  Ballantyne  is  of  the  same  opinion. 


Fio.  49. — A  section  through  the  optic  disc  to  show  cedematous  swelling 
in  a  case  of  nephritis.  Ophthalmoscopically  it  was  a  case  of  pure 
papillo3dema  with  no  exudates  or  haemorrhages  in  the  surrounding 
retina. 

The  blood  pressure  in  four  of  my  cases,  including  the  two 
referred  to,  was  200,  245,  250,  and  225. 

Retinal  Detachments. — To  judge  from  published  cases, 
detachment  of  the  retina  would  seem  to  be  a  rare  complication 
of  renal  retinitis  ;  this,  however,  is  not  the  case  if  patients  are 
examined  right  up  to  the  time  of  their  death.  Thus  I  have 
published  x  thirteen  cases  which  I  was  able  to  discover  in  the 
course  of  two -and-a -half  years. 

1  Royal  London  Ophth.  Hosp.  (Moorftelds)  Reports,  1916,  xx.,  262. 


RETINAL  DETACHMENTS 


151 


They  are  most  common  in  those  cases  in  which  the  changes 
in  the  retina  are  most  profuse,  and  are  probably  for  this  reason 
especially  common  in  pregnancy  cases  (Fig.  41,  p.  137).  In  so  far 
as  they  are  usually  associated 
with  severe  retinal  changes 
they  indicate  a  bad  prognosis, 
but  many  patients  survive, 
and  in  this  case  the  retinal 
detachments  disappear. 

Of  forty -four  cases  (thirteen 
of  my  own  and  thirty-one 
collected  cases)  seventeen 
were  associated  with  preg- 
nancy, and  of  these,  nine 
made  a  temporary  recovery  ; 
whereas  of  the  twenty-seven 
other  cases,  four  only  are 
known  to  have  recovered,  and 
seven  are  either  not  traced  or 
are  under  observation. 

The  detachments  are  due 
to  effusion  of  fluid  from  the 
retina  into  the  subretinal 
space.  They  are  usually"  bi- 
lateral, globular,  and  involve 
the  lower  part  of  the  retina 
(Fig.  41,  p.  137).  Manyofthe 
patients  are  the  subject  of 
widespread  anasarca.  I  be- 
lieve, however,  that  the  de- 
tachments are  due  to  the  local 
changes  in  the  retina,  and 


FIG.  50. — A  vertical  section  through  the 
posterior  part  of  the  eye  of  a  man, 
who,  seven  and  a  half  years  pre- 
viously, had  had  so  severe  a  renal 
retinitis  as  to  produce  a  retinal  de- 
tachment in  each  eye.  The  detach- 
ment of  each  eye,  as  seen  ophthal- 
moscopically,  disappeared  about  seven 
years  before  death,  and  there  was  no 
sort  of  residue  of  them  in  either  eye 
on  histological  examination. 


are  not  an  expression  of  the 
general  oedema,  for  in  spite  of  severe  general  oedema  in 
many  cases  of  nephritis,  acute  and  chronic,  I  have  never  seen 
retinal  detachment  in  the  absence  of  retinitis,  and  this  usually 
of  a  severe  type.  I  have,  however,  once  seen  it  in  a  case  in 
which  the  retinal  changes,  apart  from  signs  of  oedema  were 


152  RENAL   RETINITIS 

slight,  and  once  also  in  a  case  of  orthostatic  albuminuria. 
Simon  1  has  seen  a  similar  case. 

As  already  stated,  recovery  is  usual  if  the  patient  survives 
(Fig.  50).  I  have  seen  the  subretinal  fluid  completely  absorbed 
within  eight  days  of  its  discovery,  and  three  days  after  the 
removal  of  twelve  pints  of  ascitic  fluid  from  the  peritoneal 
cavity  :  this  is  the  shortest  time  in  which  I  have  seen  recovery 
occur. 

Subjective  Symptoms.— In  some  cases  of  retinitis  subjective 
visual  phenomena  are  marked.  Objects  like  flowers  or  butterflies 
are  not  infrequently  complained  of ;  in  other  cases  all  objects 
are  blue,  or  lights  and  patches  of  different  colour  are  seen. 
Simon  2  points  out  the  frequency  of  the  failure  to  recognise 
yellow  and  blue  tints. 

Very  extensive  retinal  changes  may  be  present  without  the 
visual  acuity  being  much  affected  so  long  as  the  macula  is  not 
involved. 

The  Subsidence  of  Retinitis. — In  the  ordinary  course  of  the 
progress  of  retinitis,  individual  haemorrhages  and  areas  of  woolly 
exudate  become  absorbed.  Fresh  haemorrhages  and  exudates 
however,  develop,  and  the  net  result  is  a  gradual  increase 
in  both  of  these ;  accompanying  these  changes  the  retinal 
vessels  become  engorged  and  oedema  of  the  retina]  tissues 
occurs. 

After  a  varying  length  of  time  subsidence  sets  in,  and  this  is 
true  whether  the  patient  improves  towards  temporary  re- 
covery, or  whether  he  gets  progressively  worse  and  dies,  so  that 
it  may  happen  that  at  the  time  of  death  the  retinitis  has  passed 
its  acme  and  has  begun  to  subside.  I  have  seen  this  stage 
reached  within  nine  weeks  of  the  beginning  of  the  retinitis  in  a 
young  girl,  and  in  eight  weeks  after  the  discovery  of  the  star 
figure  in  a  man  of  thirty-six.  The  progress  of  the  changes  is 
more  rapid  in  the  young. 

The  earliest  indication  that  subsidence  is  beginning  is  a 
reduction  in  the  size  of  the  veins.  During  the  progressive  stage 
they  are  engorged,  but  as  the  retinitis  begins  to  subside  they 

1  Klin.  Monats.f.  Augenheil.,  1905,  xliii.,  i,  221. 

2  Centralblatt  fiir  prakt.  Augenheil,  1894,  xviii.,  132. 


SUBSIDENCE  153 

become    smaller,    till  they   are  less   than    the   normal;    they 
develop  white  lines  along  them  near  the  disc. 

Another  change  is  seen  in  the  curves  of  the  veins;  these, 
instead  of  being  full  and  even,  tend  to  be  somewhat  angular 
or  craggy,  as  if  their  previous  distension  had  to  some  extent 
impaired  their  resilience. 


FIG.  51. — Shows  a  renal  retinitis  which  is  well  on  its  way  to  subsidence, 
as  evidenced  by  the  pale  secondary  atrophic  disc,  the  greatly 
attenuated  vessels,  especially  the  arteries,  the  sheathing  of  the  veins, 
the  powdery  nature  of  the  exudate,  and  the  pigment  spots.  From  a 
man  of  forty-six  who  had  had  so  severe  a  retinitis  as  to  produce  a 
double  detachment  of  the  retina ;  he  died  in  uraemia  six  years  after 
the  drawing  was  made. 

Changes  exactly^  corresponding  with  the  foregoing  also  take 
place  in  the  arteries,  till  ultimately  they  may  be  one-half  or  less 
of  their  normal  diameter  (Fig.  51). 

The  optic  papilla,  at  first  swollen  and  cedematous,  with 
haemorrhages  and  exudates  on  it  or  in  its  vicinity,  later  under- 
goes atrophy  and  becomes  pale  with  hazy  edges ;  it  has  the 
appearance  of  a  secondary  optic  atrophy  (Fig.  51). 


154  RENAL  RET1NITIS 

The  earliest  of  the  patches  of  exudate  to  disappear  are  the 
"  cotton  wool "  patches  ;  the  more  solid -looking  exudates  are 
more  permanent  (Fig.  53,  p.  157). 

When  subsidence  is  well  advanced  there  will  be  found 
scattered  irregularly  over  the  central  regions  a  very  fine  powdery 
white  residue,  which  has  no  regular  arrangement  even  though  a 
star  figure  was  previously  present. 

This  fine  powdery  white  residue  is  very  characteristic  of  a 
subsided  retinitis  (Fig.  51). 

Amongst  it  there  may  be  a  few  more  solid-looking  spots  or 
areas,  perhaps  with  cholesterin  crystals  in  them ;  these  persist 
for  a  long  time,  as  also  do  a  few  small  scattered  haemorrhages, 
but  if  the  patient  survives,  all  the  exudates  and  haemorrhages 
disappear  completely. 

Pigmentary  Changes. — After  subsidence,  some  pigmentary 
disturbance  is  almost  always  to  be  seen  ;  it  is  mostly  of  an 
entirely  irregular  type,  and  is  in  part  due  to  the  migration  of 
pigment  into  subretinal  exudate,  which  exudate  is  subse- 
quently absorbed. 

In  addition  to  this,  characteristic  round  or  angular  pigment 
spots  may  appear,  especially  towards  the  periphery.  They  are 
rather  uniform  in  size,  shape,  and  texture  (Fig.  51,  p.  153)  and 
have  been  figured  by  Liebreich,1  and  are  described  by  Nettle- 
ship.2  They  are  never  plentiful,  and  occur  in  loose  groups. 
I  have  not  seen  them  disappear  and  it  seems  unlikely  that  they 
would  do  so.  They  are  composed  of  wart-like  proliferations 
of  the  pigment  epithelium  (Fig.  52). 

On  differing  Types  of  Renal  Retinitis. — It  is  of  some  import- 
ance to  inquire  whether  there  are  different  forms  of  retinitis 
associated  with  different  varieties  of  kidney  disease,  or,  short 
of  this,  whether  there  are  features  of  the  retinitis  which  are 
suggestive  of  one  or  other  of  the  factors  which  are  especially 
concerned  in  nephritis. 

I  believe  the  general  feeling  of  ophthalmic  surgeons  is,  that 
a  fundus  which  shows  numerous  cotton-wool  patches,  with 
oedema  and  perhaps  a  retinal  detachment,  implies,  not  a  diffe- 

1  Atlas  Tab.,  ix.,  Figs.  1  and  2. 

2  Trans.  Ophth.  Soc.  U.K.,  1899,  xix.,  63. 


155 


rent  stage  of  disease,  but  a  disease  of  a  different  type  from  that 
which  is  implied  by  a  fundus  showing  marked  vascular  change, 
with  more  numerous  flame-shaped  haemorrhages,  little  oedema, 
a  star  figure  composed  of  discrete  dots,  and  no  "  cotton-wool  " 
patches. 

It  will,  however,  be  agreed  that  there  are  many  intermediate 
cases  which  it  is  impossible  to  classify  on  these  lines. 

It  is  believed  by  many  that  there  are  two  outstanding  factors 


ft-ttH* 

&s&i^Mf'y  ;s:« 

— i 


— B 


as^^u^  •• 


Fie;.  52. — Showing  a  wart-like  overgrowth  of  the  retinal  pigment  epithelium 
jin  a  case  of  renal  retinitis.  (See  also  Fig.  51.)  A.  Rod  and  cone 
layer.  B.  Pigment  epithelium.  C.  Choroid. 

concerned  in  nephritis,  a  toxic  and  a  vascular,  and  that  these 
are  of  varying  importance  in  the  different  types;  that  in 
parenchymatous  nephritis  the  toxic  element  prevails  and  the 
vascular  is  less  important,  in  the  arterio-sclerotic  kidney  the 
whole  disease  is  primarily  vascular  in  origin,  and  between  these 
two  extremes  is  every  grade  of  kidney  disease,  and  the  secondary 
contracted  kidney  of  Herringham  is  in  some  sense  intermediate 
between  these  two  forms. 

I  believe  cotton-wool  patches,  with  the  accompanying  oedema 
and  few  haemorrhages,  are  a  product  of  the  toxic  element  of  the 
disease ;  that  the  small  rather  scanty  discrete  hard-edged  dots, 


156  RENAL  RETINITIS 

which  may  or  may  not  form  a  star  figure  and  are  described 
under  arterio -sclerotic  retinitis,  are,  in  combination  with 
evidences  of  retinal  arterio -sclerosis,  a  pure  product  of  the 
vascular  disease,  and  that  intermediately  is  the  coarser  textured 
star  figure,  with  rather  numerous  flame-shaped  hemorrhages, 
and  a  few  larger  soft-edged  areas,  which  are  associated  with  a 
secondary  contracted  kidney. 

The  difference  in  ophthalmoscopic  appearances  between 
arterio-sclerotic  retinitis,  and  the  retinitis  associated  with 
parenchymatous  nephritis  is  so  great  that  it  would  not  be 
imagined  that  they  were  in  any  way  related. 

I  feel  confident,  that  in  no  inconsiderable  number  of  cases  the 
ophthalmoscopic  appearances  of  renal  retinitis  are  sufficiently 
distinctive  to  indicate  the  type  of  kidney  disease  which  is 
present,  but  in  many  cases  no  such  deduction  is  possible, 
and  one  may  remember  that  the  clinical  findings  of  the 
physician  in  the  same  way  are  not  always  conclusive  in  this 
direction. 

The  retinal  changes  associated  with  a  small  white  kidney 
are  perhaps  worthy  of  special  mention. 

This  form  of  disease  occurs  in  the  young,  and  is  characterised 
by  a  very  high  blood  pressure  and  absence  of  oedema. 

The  retinal  changes  are  seldom,  if  ever,  absent  ;  they  are 
severe  in  degree,  and  a  particularly  striking  star  figure  of  rather 
coarse  texture  is  generally  seen.  Rut  son  James  l  reports 
twenty-four  cases  in  detail. 

Recurrence  of  Renal  Retinitis. — When  an  eye  has  been  the 
subject  of  retinitis,  and  this  has  completely  subsided,  does  it 
recur  in  a  subsequent  recrudescence  of  the  nephritis  which 
proves  fatal  ? 

It  would  be  expected,  a  priori,  that,  unless  the  retina  was 
completely  atrophied — which  probably  never  occurs  from 
retinitis  alone — that  retinitis  would  again  develop,  but  in  a 
modified  form  ;  recurrent  haemorrhages  certainly  take  place. 
I  have  watched  several  patients  for  a  number  of  years  after 
subsidence  of  retinitis,  and  have  not  seen  any  evidence  of 
recurrence  of  it. 

1  Ophth.  Review,  1913,  xxxii.,  164. 


RECURRENCE 


157 


Knies  1  mentions  a  case  of  haemorrhagic  retinitis  in  one  eye, 
the  other  eye,  which  was  blind  from  a  previous  retro-bulbar 
neuritis,  being  spared. 


D 


FIG.  53. — A  composite  diagram  to  indicate  the  length  of  time  required  for 
the  disappearance  of  different  types  of  retinal  exudate  in  renal  or 
arterio -sclerotic  retinitis.  The  patches  of  different  types  of  exudate 
have  been  collected  from  their  proper  notes  and  incorporated  here. 
The  time  given  dates  from  the  occasion  when  the  patch  was  first 
seen.  A.  A  small  "  cotton  wool  patch  "  which  had  completely  dis- 
appeared in  five  weeks.  B.  A  small  "  cotton  wool  patch  "  which  had 
completely  disappeared  in  two  weeks.  C.  Discrete  dots  of  exudate 
in  arterio -sclerotic  retinitis ;  no  change  in  them  could  be  appre- 
ciated at  the  end  of  eleven  weeks.  D.  A  partial  star  figure  from  a 
case  of  arterio -sclerotic  retinitis.  After  thirty  weeks  two  dots  only 
were  left,  and  there  was  no  trace  of  the  condition  three  years  later. 
E.  Almost  conglomerate  exudate :  there  were  powdery  remains 
seven  months  later.  F.  Rather  solid-looking  exudate  :  it  had  dis- 
appeared five  weeks  later.  G.  Solid-looking  exudate  with  included 
cholesterin  crystals  :  it  was  still  present  seven  months  later. 

Nettleship  2  reports  a  case  in  which  one  eye  only  was  affected, 
the  unaffected  eye  being  blind  as  a  result  of  thrombosis  of  the 

1  "  Eye  in  General  Diseases,"  New  York,  1895,  306. 

2  Royal  London  OphUi.  Hosp.  Reports,  1903,  xv.,  340. 


158  HEMMIC  AMAUROSIS 

central  retinal  artery  ;  a  second  pregnancy  case,  in  which  eye 
symptoms  recurred,  but  there  was  no  fresh  retinitis  ;  and  a 
third  case,  in  which  typical  retinitis  was  present  in  the  right 
eye,  which  had  no  refractive  error,  whilst  the  left  eye  seemed 
to  be  protected  against  its  development  from  the  fact  that  it 
was  highly  myopic.  I  have  seen  a  similar  case. 

In  the  patient  previously  referred  to  (p.  135  and  Fig.  51)  a  very 
extensive  retinitis  occurred,  and,  as  has  been  explained,  com- 
plete subsidence  took  place,  leaving  the  retina  and  choroid  in  an 
atrophied  condition.  He  was  under  my  constant  observation  for 
seven  years  and  three  months,  and  died  in  hospital  of  uraemia — 
still  under  my  observation — with  a  blood  pressure  of  240  mm. 
During  the  whole  of  this  time  there  was  never  the  slightest 
evidence  of  recurrence  of  the  retinitis  up  to  the  day  before  his 
death,  at  which  time  he  was  last  examined.  I  can  feel  no 
doubt  that,  but  for  the  previous  retinitis  and  consequent 
partial  atrophy  of  the  retina,  he  would  not  have  died  in  the 
manner  he  did  without  retinitis  having  developed. 

I  believe  that  an  antecedent  disease  of  the  eye  grounds, 
whether  it  be  renal  retinitis  or  other  morbid  change,  protects 
the  eye  against  the  development  of  renal  retinitis,  and,  conse- 
quently, that  a  recurrence  of  the  retinitis  of  renal  disease  is 
quite  unusual ;  I  cannot,  however,  be  sure  that  such  protection 
is  absolute,  and  it  seems  unlikely  that  it  should  be  so. 

UR^EMIC   AMAUROSIS. 

In  some  cases  of  approaching  uraemia  loss  of  sight  occurs. 
The  characteristic  symptoms  are :  the  sudden  onset  and 
completeness  of  the  blindness,  the  rapid  and  complete  recovery, 
the  absence  of  any  corresponding  changes  in  the  retina,  and 
the  retention  of  the  pupil  reaction  to  light. 

It  is  a  striking  fact  that  in  some  cases  there  are  no  prominent 
cerebral  symptoms  of  uraemia,  so  that  such  patients  may  present 
themselves  at  the  out-patient  department  of  an  eye  clinic. 
Renal  retinitis  may  or  may  not  be  present,  and  in  the  former  case 
some  care  is  necessary  to  avoid  attributing  blindness,  which  is,  in 
fact,  of  ursernic  origin,  to  the  local  morbid  changes  in  the  retinal 


GOUT  159 

:' 

tissues,  it  will,  however,  be  readily  appreciated  how  dispropor- 
tionate is  the  loss  of  sight  to  the  local  retinal  changes. 

The  fact  that  the  pupil  reactions  to  light  are  retained 
suggests  that  the  visual  defect  is  located  in  the  cerebral  cortex. 

Lardaceous  Disease. — Of  lardaceous  disease  Herringham 
says  :  '  The  heart  does  not  hypertrophy,  the  blood  pressure 
does  not  rise,  and  albuminuric  retinitis  does  not  occur." 

Nettleship  says  that  few  cases  have  occurred,  and  that 
further  observations  are  necessary. 

I  can  only  say  that  I  have  found  entirely  normal  fundi  in 
the  few  cases  that  I  have  examined. 

GOUT. 

So  numerous  are  the  manifestations  attributed  to  that 
disorder  of  metabolism  to  which  the  term  constitutional  gout 
is  applied,  that  it  need  cause  no  surprise  to  find  that  at  different 
times,  by  different  observers,  a  gouty  form  of  many  of  the 
diseases  of  the  eye  has  been  described  ;  and  whilst  so  many 
forms  of  eye  disease  with  cause  unknown  remain,  it  is  not 
possible  to  deny  that  so  protean  a  disease  may  at  times  be  justly 
held  responsible  for  some  of  them. 

At  the  same  time  the  evidence  of  causal  relationship  is 
frequently  of  the  slenderest,  and  it  would  often  seem  less 
retrograde  to  uncover  one's  ignorance  under  so  discredited  a 
term  as  "  idiopathic  "  than  to  apply  a  title  which,  too  often,  is 
but  a  placebo  to  the  patient,  and  to  the  surgeon  a  self-deception. 

In  very  few  instances  are  there  distinctive  characteristics 
which  enable  one  to  diagnose  a  gouty  origin  for  any  disease  of 
the  eye. 

Of  the  lesions  whose  relationship  to  this  disease  seem  best 
attested  the  following  may  be  mentioned  : — 

First,  a  group  of  cases  which  is  associated  with  disease  of 
the  vessels  has  been  described  under  terms  such  as  "  hsemor- 
rhagic  retinitis,"  "  phlebitis,"  "  venous  thrombosis,"  phlebo- 
sclerosis,"  "  arterio-sclerosis,"  etc.,  by  Hutchinson,1  Gale- 
zowski,2  Spillman,3  Bull,  and  many  others  ;  these  have 

1  Roy.  Land.  Ophth.  Hosp.  (Moorfields)  Reports,  1873,  iii.,  315,  455. 

2  Becueil  cFOphth.,  1900,  xxii.,  65. 

3  Gaz.  hebd.  de  med.,  1890,  xxvii.,  480. 


160  GOUT 

already  been  dealt  with  under  the  head  of  vascular  disease, 
without  prejudice  to  the  relationship  of  gout  to  them. 

Iritis  is  seen  not  infrequently  in  patients  at  or  beyond  middle 
age  in  whom  none  of  the  more  usual  causes  are  found.  In  some 

o 

such  cases  joint  pains  are  complained  of,  and  the  condition  is 
often  attributed  to  gout. 

Hutchinson  1  describes  gouty  iritis  as  occurring  in  two  forms. 
One  especially  affects  males  in  vigorous  health  past  middle 
age  ;  first  one  eye  is  affected,  then  the  other,  but  seldom  both 
together.  The  other  form  occurs  most  often  in  women,  both 
eyes  being  commonly  affected,  and  the  ciliary  body  and  choroid 
are  frequently  implicated.  He  states,  that  of  104  cases  of 
iritis,  a  positive  history  of  gout  was  obtained  in  eighteen,  and 
in  twenty-nine  there  was  a  positive  history  of  gout  in  near 
relatives,  i.e.,  unequivocal  gout  in  the  big  toe. 

In  other  cases  of  persistently  recurring  iritis,  cyclitis,  and 
scleritis  in  middle-aged  patients,  in  which  corneal  opacities 
and  ciliary  staphylomata  often  arise,  Hutchinson  says  :  "  It  is 
probable  that  in  some  ca"ses  the  constitutional  cause  of  this 
destructive  and  persisting  disease  is  gout." 

Conjunctivitis. — An  obstinate  form  of  recurrent  conjunctivitis 
is  recognised  by  many  as  associated  with  gout. 

This  was  described  by  Hutchinson  under  the  term  of  "  hot 
eye."  He  speaks  of  it  as  a  common  phenomenon  which  attends 
quiet  gout.  It  is  characterised  by  short  attacks  of  congestion 
which  sometimes  affects  both  eyes.  The  conjunctiva  becomes 
red  and  the  eyeball  feels  hot,  and  pricks  as  if  sand  were  in  it. 
It  may  last  a  few  hours  or  a  day  or  two,  and  not  infrequently 
culminates  in  iritis. 

Of  other  lesions  it  is  greatly  to  be  doubted  whether  gout 
ever  produces  the  ophthalmoscopic  signs  of  optic  neuritis,  or 
is  responsible,  except  through  the  intermediation  of  vascular 
disease,  for  ocular  paralysis. 

Gowers  2  states,  however,  that  spontaneous  inflammation  of 
nerve  trunks  and  plexuses  on  one  side  only,  and  recurring  after 
the  age  of  thirty,  are  seldom  due  to  any  cause  other  than 

1  Bowman  Lecture,  Trans.  Ophth.  Soc.  U.K.,  1885,  v.,  <J. 

2  Med.  Ophthalmoicopy,  1934,  254. 


DIABETES  161 

gout.  He  believes  that  inflammation  of  the  sheath  of  the  optic 
nerve  behind  the  eye  is  less  common  in  ordinary  gout  than 
inflammation  in  the  retina.  As  characteristic  of  these  affections 
he  gives  the  greater  degree  of  disturbance  of  vision  than 
corresponds  with  the  visible  changes  in  the  optic  discs,  the  ten- 
dency to  irregular  defects  in  the  fields  of  vision,  and  the  strong 
tendency  for  the  other  optic  nerve  to  be  affected  by  an 
independent  symmetrical  morbid  process. 

DIABETES. 
Diabetic   Retinitis. 

Doubt  has  been  expressed  as  to  whether  a  retinitis  due  to 
diabetes  alone  ever  occurs.  The  most  weighty  reason  for 
doubting  the  existence  of  a  pure  diabetic  retinitis  is,  that  in 
young  subjects,  in  whom  the  disease  manifests  itself  in  its  most 
malignant  form,  and^in  whom  the  toxic  elements  are  most 
prominent,  retinitislieTeiroccurs. 

A  second  reason  which  lends  weight  in  the  same  direction  is 
the  fact,  that  it  is  usual  to  find  albumen  as  well  as  sugar  in  the 
urine  of  patients  with  diabetic  retinitis  ;  and  further,  it  is 
quite  unusual  to  come  across  such  a  patient  who  has  not  also 
a  considerably  heightened  systolic  blood  pressures 

It  is  evident  in  the  face  of  these  facts,  that  the  reasoned  doubts 
expressed  by  Sir  Archibald  Garrod  1  require  to  be  considered. 

Reasons  for  Differentiating  Diabetic  Retinitis  as  a  Separate 
Entity. — (1)  There  is  a  strong  family  likeness  between  diabetic, 
renal,  and  arterio -sclerotic  retinitis,  and  there  are  few  indi- 
vidual cases  in  which  it  is  possible  to  state  dogmatically  from 
an  ophthalmoscopic  examination  alone,  that  a  retinitis  which 
is  present  is  due  to  diabetes. 

At  the  same  time  there  are  features  which,  whilst  they  are 
not  pathognomonic,  when  combined,  form  a  picture  which 
suggests  diabetes  as  the  probable  cause,  and  little  more  than 
this  can  be  said  of  renal  retinitis,  though  the  difficulty  is  much 
less  (Fig.  54). 

These  features  are  : — 

(a)  The  patches  of  retinal  exudate  in  diabetes  tend  to  have 
1  Trans.  Ophth.  Soc,  U.K.,  1920,  xl.  6. 


162 


DIABETES 


sharp-cut  edges  ;   are  often  solid  and  soapy  or  waxy- 
looking  ;  are  usually  distributed  in  an  irregular  manner, 
and  sometimes  form  an  irregular  ring  well  wide  of  the 
macula  (Fig.  54). 
(b)  A  star  figure  is  uncommon,  and  if  present  it  does  not 


FIG.  54.- — From  a  woman  of  fifty-seven,  the  subject  of  diabetes.  Show- 
/  ing  the  rather  solid  waxy  or  soapy -looking  exudate  distributed 
roughly  in  a  ring  rather  wide  of  the  yellow  spot ;  there  is  no  tendency 
to  the  formation  of  a  star  figure.  The  haemorrhages  are  small  and 
more  or  less  circular  in  shape  ;  flame-shaped  haemorrhages  are  almost 
absent. 

acquire  the  degree  of  symmetry  that  may  be  seen  in 
renal  cases. 

(c)  The  soft-edged  cotton-wool  patches,  so  frequent  in  severe 

renal  cases,  do  not  appear  in  diabetes  ;  retinal  oedema 
is  never1  so  marked,  and  thus  retinal  detachment  does 
not  result. 

(d)  Retinal  haemorrhages  are  generally  in  the  deeper  retinal 


DIABETIC  RET  I  NIT  IS  163 

layers,  and  therefore  are  roughly  circular  in  outline 
instead  of  being  flame-shaped. 

(e)  The  circular  retinal  pigment  spots  which  are  not  rare  in 
the  later  stages  of  renal  retinitis  are  not  seen,  I  think, 
in  diabetes. 

(2)  As  has  been  said,  patients  with  diabetic  retinitis  usually 
have  a  heightened  blood  pressure,  and  albumen  in  the  urine 
if  this  be  repeatedly  examined. 

A  number  of  cases  have,  however,  been  recorded  in  which 
the  urine  was  albumen-free  throughout  the  disease.  Nettle- 
ship  states  x  that  he  has  seen  at  least  twelve  such  cases.  Kako2 
found  albumen  in  twenty-one  cases  only  out  of  fifty-seven  cases 
of  diabetic  retinitis.  Other  such  cases  are  reported  by  Stephen 
Mackenzie,3  James  Anderson,4  Henry  Juler,5  Rockliffe,6 
Hirschberg.7 

/~"  I  have  seen  what  I  believed  to  be  a  typical  diabetic  retinitis 
in  a  man  of  sixty-three,  who  had  both  albumen  and  sugar  in 
his  urine,  and  whose  systolic  blood  pressure,  taken  at  weekly 
intervals,  never  exceeded  155  mm.  ;   it  is  exceedingly  unusual  • 
to  find  either  renal  or  arterio -sclerotic  retinitis  with  a  blood 

pressure  so  little  removed  from  normal  as  this.     His  blood 

-.    ,  . 

sugar  content  was  so  low  as  0-05  per  cent.  4,  /  <-/  +t 

I  have  seen  on  several  occasions  diabetics  between  the  ages 
of  forty  and  fifty  with  retinitis,  in  whom  the  blood  pressure 
was  not  at  all  raised,  and  the  urine  was  free  of  albumen. 

I  think  it  may  be  said,  on  the  one  hand,  that  the  combination 
of  a  normal  blood  pressure  with  urine  free  of  albumen  is  in- 
compatible with  a  diagnosis  of  renal  or  arterio-sclerotic  retinitis, 
and  when  instead  the  patient  is  suffering  from  diabetes,  it 
would  seem  hard  to  deny  that  the  retinitis  is  indeed  due  to 
this  latter  diseased 

1  Royal  Lond.  Ophth.  Hasp.  Reports,  1903,  xv,  338;   Trans.  Ophlh.  Soc.  U.K., 
1899,  ix.,  160. 

2  Klinisch.  Monats.  f.  Augen.,  1903,  xli,  257. 

3  Royal  Lond.  Ophth.  Hosp.  Reports,  1879,  ix.,  136. 

4  Ophth.  Review,  1889,  viii.,  33. 

5  Trans.  Ophth.  Soc.,  1892,  xii.,  137. 
8  Trans.  Ophth.  Soc.,  1894,  xiv.,  130. 

7  Centrdblattf.  prak.  Augenheilk.  1891,  xv.,  18,  68. 


164 

(3)  The    immediate    cause    of    death    was    ascertained    by 
Nettleship  x  in  twenty-four  patients  who  had  diabetic  retinitis. 
Eleven  of  them  died  in  diabetic  coma  and  four  died  of  gangrene. 

(4)  The  prognosis  (vide  infra)  is  much  less  grave  than  in 
renal  cases. 

(5)  In  its  age  limit  it  contrasts  markedly  with  renal  retinitis. 

(6)  It   is   not   infrequently   unilateral,    and-  whilst   arterio- 


Fio.  55. — A  section  through  the  retina  showing  diabetic  retinitis.  The 
fibrinous  exudate  is  almost  confined  to  flask-like  cavities  in  Henle's 
layer.  A.  Rod  and  cone  layer. 

sclerosis  is  usually  present,  it  has  a  number  of  the  characters 
which  distinguish  it  from  arterio -sclerotic  retinitis. 

It  is  evident  that  many  patients,  being  diabetics,  develop  a 
retinitis,  which,  but  for  the  disease  would  not  have  occurred, 
and  whilst  concomitant  tissue  changes  may  be  wholly  or  partly 
responsible  for  the  development  of  the  retinitis,  these  changes 
themselves  are  presumably  dependent  upon  the  diabetes. 

There  seems,  then,  to  be  good  reason  for  identifying  a  special 
form  of  retinitis  due  to  diabetes. 

It  is  evident  that  neither  the  products  of  perverted  meta- 

1  Royal  Land.  Ophth.  Hasp.  Repbrts,  1906,  xvi.,  14. 


DIABETIC  RETINITIS  165 

bolism,  di-acetic  acid  and  acetone,  nor  hyperglycsemia,  are 
responsible  for  the  development  of  the  fundus  disease,  for 
young  subjects  in  whom  these  manifestations  are  most  advanced 
are  free  from  retinitis. 

Incidence. — Age. — Retinitis  affects  older  patients  only. 
Nettleship  states  *  that  of  fifty-nine  patients  of  his  own  and 
twenty-three  published  cases,  eighty-two  in  all,  the  youngest 
was  thirty -five.  Of  sixty-two  patients  of  my  own  2  the  youngest 
was  thirty-nine. 

It  may  be  said  for  practical  purposes  that  diabetic  retinitis 
does  not  occur  under  the  age  of  thirty -five. 

It  is  not  easy  to  form  an  estimate  as  to  the  frequency  of 
the  condition,  for  in  a  general  hospital  the  majority  of  diabetic 
patients  who  are  admitted  to  the  wards  are  the  young,  in 
whom  retinitis  does  not  occur,  whereas  those  who  attend  an 
ophthalmic  hospital  come  only  because  their  sight  is  defective, 
and  consequently  include  many  cases  of  retinitis. 

Of  fifty-one  patients  of  all  ages  admitted  to  St.  Bartholo- 
mew's Hospital  five  only  had  retinitis.  Kako  found  fifty-seven 
cases  amongst  192  cases  of  diabetic  eye  affection. 

Prognosis. — The  prognosis  is  not  nearly  so  grave  as  in  renal 
retinitis,  nor  so  grave  as  in  artero -sclerotic  retinitis.  Of  forty- 
eight  patients  kept  under  observation  by  Nettleship,3  thirty- 
eight  were  known  to  have  died,  and  ten  were  known 
to  be  alive  at  periods  varying  from  two  to  ten  years 
after  the  discovery  of  the  retinitis.  Of  the  thirty-eight, 
nineteen  died  within  two  years  of  the  discovery  of  the  retinitis  ; 
the  remaining  nineteen  died  within  a  period  varying  from  two 
to  eight  years.  Thus,  of  forty-eight  patients,  60  per  cent, 
lived  for  longer  than  two  years  after  retinitis  was  found  to  be 
present. 

Haemorrhages.— Apart  from  the  presence  of  other  changes, 
diabetics  are  especially  liable  to  intra-ocular  haemorrhages. 

These  may  occur  amongst  the  layers  of  the  retina,  and  if 
confined  to  this  structure  are  readily  visible  ophthalmoscopi- 

1  Royal  Lond.  Ophth.  Hasp.  Reports,  1903,  xv.,  339. 

2  Trans.  Ophth.  Soc.  U.K.,  1920,  xl.,  16. 

3  Royal  Lond.  Ophth.  Hosp.  Reports,  1906,  xvi..  11. 


166 


DIABETES 


cally,  and  will  have  characteristic  features,  according  as  the 
nerve  fibre  layer  or  the  deeper  layers  are  concerned.  Some- 
times all  the  retinal  layers  are  widely  infiltrated,  at  other 
times  haemorrhages  of  the  subhyaloid  type  occur,  or  again, 
bleeding  may  take  place  into  the  vitreous  body  and  may 


F:G.  56. 


FIG.  57. 

To  [show  the  condition  of  the  pigment  epithelium  on  the  posterior 
surface  of  the  iris  in  a  case  of  diabetes,  and  in  a  normal  eye. 
Fig.  56  shows  the  normal  condition  of  the  epithelium,  Fig.  57 
the  condition  of  this  layer  in  diabetes.  A.  Pigment  epithelium. 
B.  Cornea.  C.  Ciliary  body. 

be  seen  as  a  smoky  cloud,  or  may  entirely  obscure  the  view  of 
the  fundus.  Occasionally  massive  craggy  white  areas  are  to  be 
seen  which  are  clothed  by  the  retina,  the  retina  itself  being  free  of 
haemorrhages,  the  condition  is  not  by  any  means  a  retinitis  ; 
it  seems  likely  that  these  areas  are  due  to  haemorrhages  of  the 
choroid,  but  of  this  I  know  of  no  proof  or  supporting  evidence. 
As  showing  the  liability  of  diabetics  to  retinal  haemorrhages, 


LIP  MM  I A    RETINALIS  167 

Schweigger  x  found  that  of  45,000  patients,  450  had  retinal 
haemorrhages  with  or  without  retinitis  ;  of  these  rather  more 
than  one-fifth  were  diabetics,  and  of  them,  those  with  haemor- 
rhages only  were  three  times  as  common  as  those  with  retinitis. 

Iritis. 

Having  regard  to  the  almost  constant  presence  of  marked 
histological  changes  in  the  epithelium  on  the  posterior  surface 
of  the  iris  in  diabetic  eyes  (Fig.  57),  it  is  remarkable  that  clinical 
evidence  of  iritis  is  so  infrequent. 

When  it  occurs  a  gelatinous 'exudate  may  form  in  the  anterior 
chamber  somewhat  like  that  seen  in  a  primary  gonorrhceal 
iritis,  but  lacking  the  yellow  tinge ;  it  rapidly  and  completely 
disappears  under  treatment. 

The  changes  in  the  pigment  epithelium  have  been  described 
by  a  number  of  observers. 

Kako  describes  a  great  enlargement  of  the  pigment  epithelial 
cells  with  reduction  in  the  pigment.  A  considerable  thickening 
of  the  iris  results  with  exaggeration  of  its  normal  folds.  The 
changes  stop  short  of  the  pupillary  margin  of  the  iris,  but 
extend  for  a  short  distance  into  the  ciliary  body.  He  states 
that  of  twenty-seven  reported  examinations,  these  changes 
were  present  in  twenty-five.  Hoffmann  2  states  that  the 
epithelium  is  proliferated,  is  deficient  in  pigment,  and  is  laden 
with  glycogen.  Becker  and  Kamocki  found  that  the  process 
extended  to  the  retina., 

Lipaemia  Retinalis. 

This  term  is  applied  to  an  exceedingly  characteristic  ophthal- 
moscopic  picture  which  occurs  in  some  cases  of  Iipa3mia,  and 
was  first  described  by  Heyl.3 

Pathological  lipaemia  occurs  in  a  number  of  disea:ros,  but  only  - 
in  diabetes  does  it  attain  a  sufficiently  high  degree  to  manifest 
itself  to  ophthalmoscopic  examination. 

Heine  believes  that  the  fat  content  of  the  blood  must  reach 

1  Inaug.  Dissert.,  Berlin,  1897. 

2  Archive  of  Ophth.,  1914,  xlii.,  41. 

3  Trans.  Amer.  Ophth.  Soc.,  1880,  iii.,  54. 


168  DIABETES 

5  per  cent  before  it  is  seen.  Thorner  reports  a  case  in  which 
the  blood  contained  26  per  cent,  of  fat. 

In  the  early  days  of  the  war  I  examined  a  series  of  119  men 
suffering  from  "  trench  nephritis  "  as  to  their  eye  condition.1 
Of  these,  four  had  well-marked  lipsemia  or  lecithinsemia,  but  in 
none  of  them  was  it  sufficiently  marked  to  give  rise  to  the 
ophthalmoscopic  picture  of  "  lipsemia  retinalis." 

The  number  of  recorded  cases  is  few,  but  if  all  young 
diabetics  who  are  approaching  coma  be  examined,  it  will  not 
be  long  before  the  searcher  is  rewarded. 

No  case  has  been  recorded  in  an  old  patient ;  the  oldest  I 
have  been  able  to  find  is  reported  by  Darling  2  in  a  man  of 
forty -eight.  My  own  patients  were  aged  twenty -three,  twenty- 
five  and  twenty -five.3 

I  have  watched  the  gradual  evolution  of  lipsemia  retinalis  in 
the  course  of  six  weeks,  in  a  man  who  was  known  to  have 
lipsemia,  but  who  did  not  at  first  show  any  signs  in  the  retina. 

The  first  indication  was  a  slightly  milky  or  hazy  appearance 
of  the  retinal  vessels,  their  colour  otherwise  remaining  normal  ; 
slowly  this  milkiness  became  more  pronounced  and  the  colour 
became  more  ctlerry  red.  The  arteries  and  veins  approximated 
each  other  in  colour  until  ultimately  the  fully  developed  picture 
occurred  shortly  before  death.  In  the  fully  developed  condi- 
tion both  arteries  and  veins  for  the  whole  of  their  extent  have 
an  appearance  as  if  filled  with  milk  ;  but  short  of  this,  in 
some  cases  the  vessels  on  the  disc  have  a  cherry  red  or  salmon 
colour,  merging  into  cream  shortly  after  leaving  its  edge.  Both 
sets  of  vessels  look  flat  and  ribbon-like  ;  they  appear  to 
lose  their  cylindrical  contour,  thus  simulating  the  normal 
appearance  of  the  choroidal  vessels.  The  veins  are  often 
engorged,  sometimes  markedly  so.  The  disc  edges  remain  sharp 
and  clear,  neither  haemorrhages  nor  exudates  occur,  and  the 
vision  is  unaffected. 

It  will  be  seen  that  the  changes  are  entirely  limited  to  the 
appearance  of  the  retinal  vessels.  The  condition  is  most 

1  Lancet,  1915,  ii.,  1348. 

2  Archives  of  Ophth.,  1912,  xli.,  355. 

3  lancet,   1915,  i.,  367  j   Tram.  Ophth.  Soc.  U.K.,  1920,  xl..  21. 


ALTERATIONS  OF   REFRACTION  169 

striking  ;  is  pathognomonic,  and  cannot  be  confused  with  any 
other  ophthalmoscopic  picture. 

Death  usually  follows  shortly  upon  the  discovery  of  the 
condition,  but  Hale  White  l  and  Marx  have  each  reported  a 
case  in  which, recovery  ensued,  and  the  eye  fundus  again  became 
normal  in  appearance.  I  have  seen  a  case  in  which  the  partially 
developed  appearances  disappeared  completely  under  treat- 
ment in  the  course  of  a  week. 

Sections  of  the  retina  and  choroid  of  my  last  case  showed  the 
vessels  filled  with  blood  the  plasma  of  which  was  well  stained 
by  Sudan  III.,  but  only  in  a  few  places  was  any  substance 
stained  by  osmic  acid. 

Alterations  of  Refraction. 

A  number  of  observers  have  reported  rapid  alterations  of  the 
refraction  of  the  eye,  due,  it  is  presumed,  to  alteration  in  the 
refractive  power  of  the  lens,  and  varying  with  the  sugar  output. 
In  most  cases  there  has  been  an  alteration  towards  myopia 
with  increase  of  sugar  output,  and  a  rapid  return  towards  the 
normal  with  a  decreased  sugar  output.  The  alterations  have 
been  as  much  as  seven  dioptres.  This  is  clearly  a  possibility  to 
have  in  mind  in  connection  with  the  development  of  amblyopia 
in  diabetics. 

Grimsdale  2  reported  the  case  of  a  man  who  rapidly  developed 
two  dioptres  of  myopia  with  return  to  the  normal  in  ten  days. 
Other  cases  are  reported  by  Risely 3 ;  Gould 4  collected 
twenty -seven  cases,  in  fourteen  of  which  there  was  an  alteration 
towards  myopia,  and  Lundsgaard  5  records  a  case  in  which 
there  was  an  alteration  of  seven  dipotres. 

L^^f^'^ 

Difficulty  with  near  vision  is  a  frequent  complaint  of 
diabetics  ;'  it  is  a  result  of  defective  power  of  accommodation 
due  to  the  general  debility  resulting  from  the  disease. 

Cataract. 

Cataract  occurs  in  two  chief  forms  in  diabetes  ;  the  one 
forming  rapidly  in  young  subjects,  the  other  a  much  more 

1  Lancet,  1903,  ii.,  1007. 

2  Trans.  Ophth.  Soc.,  1899,  xix.,  267. 

8  Trans.  Amer.  Oph.  Soc.,  1897,  viii.,  121. 

*  Med.  Record,  1907,  Ixxi.,  633. 

5  Klin.  Monats.f.  Aug.,  1910,  xlviii.,  2,  38. 


170 


DIABETES 


slowly  developing  form  in  old  patients.  The  term  "  diabetic 
cataract  "  is  best  restricted  to  the  former  of  these. 

The  characteristics  of  this  variety  are,  that  it  develops 
rapidly^  usually  commences  in  the  posterior  layers  of  the  lens, 
is  of  a  homogeneous  texture,  is  bilateral,"  and  occurs' only  when 
the  disease  is  well  advanced.  In  many  cases  the  condition  of 
the  patient  is  such  as  to  make  operation  for  the  condition 
inadvisable,  but  in  some  cases  removal  is  desirable,  and 
James  1  has  shown  what  good  results  are  usually  obtained. 

Of  my  cases  of  diabetes  under  the  age  of  forty,  twenty-nine 
in  number,  two  only  had  cataract.  In  diabetics  over  the  age 
of  fifty  opacities  are  much  more  common  ;  it  has  to  be  remem- 
bered, however,  that  at  and  beyond  this  age  senile  lens  opacities 
are  of  very  frequent  occurrence,  and  are  increasingly  common  as 
age  advances,  until  at  the  age  of  sixty,  about  90  per  cent,  of  all 
subjects  have  some  degree  of  opacity  in  the  lens. 

There  is  nothing  in  the  appearance  of  the  lens  opacity  in  these 
patients  to  distinguish  it  from  an  ordinary  senile  cataract, 
though  probably  it  is  true,  that  the  frequency  of  cataract  in 
older  diabetics  is  greater  than  in  other  patients  of  the  same  age 
without  diabetes.  Kako  found  eighty-four  cases  amongst  194 
patients  suffering  from  diabetic  eye  affections ;  he  expressly 
states,  however,  that  the  number  includes  senile  cataracts 
occurring  in  diabetic  patients. 

This  form  of  cataract  often  calls  for  removal,  and  whilst  the 
healing  is  often  less  smooth  and  less  free  from  complications 
than  in  uncomplicated  senile  cases,  the  majority  do  quite  well. 
Sir  Archibald  Garrod  recommends  that  a  preliminary  dietetic 
regime  on  Graham's  lines  should  always  precede  operation  for 
at  least  two  weeks.  Burdon  Cooper  2/Has  shown  that  in  senile 
cataract  the  lens  albumen  is  broken  down  "fco  tyfosin,  and  he 
suggests  that  in  diabetic  cases  cholesterin  may  be  formed  and 
be  responsible  for  the  opacity^ 

Retro-bulb ar  Neuritis. — Without  objective  disease  in  any  part 
of  the  eye,  a  certain  number  of  diabetics  complain  of  defective 
vision.  Some  of  these  are  no  more  than  a  failure  of  the  ciliary 

1  Trans.  Ophth.  Soc.  U.K..  1920,  xl.,  40. 

2  Trans.  Ophth.  Soc.  U.K.,  1920,  xl.,  45. 


RETEO-BULBAR  NEURITIS  171 

muscle  owing  to  general  debility,  producing  difficulty  in  reading 
for  the  emmetrope,  and  general  amblyopia  for  the  hyperme- 
trope.  Some,  however,  are  due  to  a  retro-bulbar  neuritis.  It 
occurs  in  middle-aged  patients,  and  the  onset  may  be  rather 
sudden. 

Of  sixty-one  diabetic  patients  of  all  ages  this  condition  was 
present  in  six.  The  average  age  was  thirty -six  the  oldest  being 
fifty-three,  in  none  was  there  any  discoverable  disease  of  the 
eye  to  account  for  the  loss  of  sight.  Leber  1  has  seen  fourteen 
cases  amongst  fifty  cases  of  diabetes.  I  have  had  a  man  brought 
to  hospital  because  he  could  only  dimly  see  people  walking. 
I  sent  a  man  home  from  France  for  this  condition  and  saw 
him  again  two  years  later ;  he  was  an  advanced  diabetic,  his 
discs  showed  a  mild  degree  of  atrophy,  and  in  addition  to  an 
absolute  central  scotoma  he  had  no  appreciation  of  colour,  and 
had  a  large  nasal  defect  in  his  visual  fields. 

A  patient  who  had  been  in  hospital  for  a  number  of  weeks  for 
diabetes  awoke  one  morning  to  find  everything,  both  near  and 
distant,  was  misty  ;  he  was  unable  to  read,  whereas  the  night 
before  he  was  reading  a  book  without  trouble  ;  he  was  both 
acetone  free  and  sugar-free  at  the  time ;  he  completely  recovered 
in  the  course  of  ten  days. 

/  There  can  be  no  doubt  that  diabetics  are  more  vulnerable  to 
the  toxic  effects  of  smoking  than  are  healthy  patients,  and  it  is 
difficult  at  times  to  determine  which  of  the  two,  the  diabetes 
or  the  tobacco,  is  the  real  factor  in  the  production  of  the 
neuritis. 

Thus  Kako  2  states  that  of  sixteen  cases,  in  two  only  could 
alcohol  and  tobacco  be  excluded  as  a  cause.  On  the  other  hand, 
Reber  3  reports  a  case  which  persisted  for  two  years,  the  woman 
being  a  non-smoker.  I  have  seen  it  in  a  woman  who  denied 
smoking,  and  have  watched  it  develop  in  a  man  whilst  he  was 
in  hospital,  where  smoking  was  not  permitted. 

If  the  neuritis  is  prolonged  some  degree  of  permanent  atrophy 
results,  as  was  shown  histologically  by  Lawford  and  Edmunds,4 

1  Bericht.  der.  ophth.  Gesells.,  Heidelberg,  1896,  xxv.,  104. 
*  Klinische  Monats.  f.  Augen.,  1903,  xli.,  253. 

3  Ophth.  Record,  1910,  xix.,  124. 

4  Trans.  Ophth.  Soc.  U.K.,  1883,  iii.,  160. 


JUx'* 

172    /yi  GRAVES'   DISEASE 

,  and  Schmidt  Rimpler.1     Its  development  does  not  appear  to 
indicate  an  increase  in  gravity  of  the  prognosis. 

Ocular  Tension. — It  has  been  pointed  out  by  Heine,2  that 
in  some  cases  of  diabetic  coma  there  is  a  remarkable  diminution 
in  the  intra-ocular  tension  which  is  readily  appreciable  to  the 
touch  ;  it  is  not  caused  by  a  corresponding  fall  in  the  blood 
pressure. 

EXOPHTHALMIC   GOITRE. 

The  ocular  symptoms  of  this  disease  are  both  striking  and 
important,  and  few  cases  occur  in  which  some  of  them  are  not 
present ;  they  are,  however,  entirely  limited  to  the  exterior  of 
the  eye,  and  its  appendages  ;  retinal,  pupillary,  or  other 
intra-ocular  changes  do  not  occur.  <j>  f_<^  \-o- 

Von  Graefe's  Sign. — This  sign  consists  in  the  failure  of  the 
upper  lid  to  follow  the  eyeball  on  looking  downwards.  Von 
Graefe  himself  looked  upon  it  as  one  of  the  early  signs  of  the 
disease,  others  have  looked  upon  it  as  of  late  occurrence. 

Hill  Griffith  3  has  described  a  lagging  behind  of  the  lower  lid 
on  looking  upwards. 

The  frequency  of  occurrence  of  von  Graefe's  sign  is  given  by 
Dock  as  50  per  cent,  of  cases.4  Mannheim  5  found  it  in  eighteen 
out  of  forty-one  cases,  and  Murray  in  thirty-six  out  of  ninety- 
one  cases.  Sharkey  6  has  shown  that  it  is  not'limited  to  cases  of 
Graves'  disease,  but  that  of  613  cases  of  all  kinds  of  diseases, 
excluding  Graves',  it  was  present  in  twelve  cases,  and  that  many 
people  can  produce  it  voluntarily. 

It  is  quite  independent  of  protrusion  of  the  eye,  for  it  may  be 
present  in  the  entire  absence  of  proptosis  ;  it  may  disappear,  the 
exophthalmos  remaining  ;  and  von  Graefe  states  he  once  saw 
it  disappear  after  an  injection  of  morphia,  the  exophthalmos 
being  unchanged. 

Dairy mple's  Sign. — This  sign  consists  in  a  retraction  of  the 

1  Benefit  der  ophth.  Gesellsch.,  Heidelberg,  1896,  xxv.,  99. 

2  Klin.  Mortals,  f.  Augen.,  1906,  xliv.,  ii.,  451. 

3  Trans.  Ophth.  Soc.  U.K.,  1886,  vi.,  61. 

4  Osier's  "  System  of  Med.,"  1915,  iv.,  863. 

5  "Der  Morbus  Gravesii,"  Berlin,  1894,  75. 
«  Lancet,  1890,  ii.,  877, 


EXOPHTHALMOS  173 

upper  lid,  producing  a  staring,  frightened  appearance,  which  is 
very  characteristic. 

According  to  Sattler  this  sign  was  first  pointed  out  by 
Dalrymple,  and  afterwards  carefully  studied  by  Stellwag  whose 
name  is  sometimes  attached  to  it. 

It  is  a  very  frequent  sign  ;  Hill  Griffith  found  it  in  nineteen 
out  of  twenty- two  cases  in  which  it  was  looked  for.  It  is  said  to 
come  on  early  (Dock). 

Stell wag's  Sign. — By  this  is  meant  marked  infrequency  and 
incompleteness  of  the  blinking  reflex,  a  condition  which  predis- 
poses to  the  development  of  corneal  ulceration  and  conjunc- 
tivitis ;  it  may  be  associated  with  a  partly  dulled  corneal 
sensation. 

By  some,  Stellwag's  name  is  attached  to  a  combination  of  the 
above  with  what  has  here  been  described  as  Dalrymple's  sign. 

Moebius'  Sign. — This  consists  in  a  difficulty  of  convergence  of 
the  eyes  on  near  objects,  and  is  quite  independent  of  muscular 
paralysis  ;  it  is  said  to  be  independent  of  the  mechanical  effects 
of  the  proptosis. 

It  is  not  a  sign  of  great  importance  for  severe  proptosis  from 
any  cause  will  produce  some  difficulty  with  convergence. 

Exophthalmos. — This  constitutes  one  of  the  three  classical 
signs  of  the  disease,  and  according  to  Dock  it  occurs  in  about 
70  per  cent,  of  cases.  Wilbrand  and  Saenger  l  found  it  in 
twenty-seven  out  of  thirty-nine  cases. 

The  eye  is  pushed  directly  forwards,  a  point  which  helps  to 
distinguish  the  condition  from  the  proptosis  caused  by  some 
intra-orbital  growths.  As  a  rule  both  eyes  are  affected,  but  it 
is  common  to  find  one  more  prominent  than  the  other,  and  in 
the  early  stage  one  eye  may  commence  to  be  prominent  before 
any  noticeable  change  is  present  on  the  other  side.  Of  thirty- 
two  cases  Hill  Griffith  found  unilateral  proptosis  in  22  per  cent., 
and  Wilbrand  and  Saenger  state  that  four  of  their  twenty-seven 
cases  were  unilateral. 

The  prominence  may  vary  in  degree  from  time  to  time,  and 
it  is  stated  that  its  onset  may  be  rapid ;  thus,  Falta  2  says 

1  "  Die  Neurologie  des  Auges.,"  1900,  i.,  50. 

2  "Die  Kikrankungen  der  Blutdrussen,"  Berlin,  1913,  50. 


174  GRAVES'   DISEASE 

exophthalmos  may  even  come  on  in  one  night  or  in  a  few  days, 
and  Dock  l  says  it  "  may  come  on  in  a  few  days  or  even  minutes, 
but  usually  does  so  slowly."  There  is  no  doubt  that  the 
proptosis  is  usually  of  comparatively  slow  development,  and  a 
sudden  development  of  it  is  excessively  rare  if  ever  it  occurs. 

Great  variations  in  the  degree  of  the  proptosis  occur,  the 
eyes  may  be  pushed  so  far  forwards  that  the  lids  are  no  longer 
able  to  close  over  the  corneee,  and  indeed,  the  globe  may  be 
dislocated  in  front  of  the  lids. 

Even  when  the  exophthalmos  is  not  very  severe,  conjuncti- 
vitis and  corneal  ulcers  are  common  on  account  of  the  unusual 
exposure,  and  are  probably  further  predisposed  to  by  the 
reduced  frequency  of  blinking,  but  in  cases  where  the  lids  are 
unable  to  meet,  severe  corneal  ulceration  is  greatly  to  be  feared, 
and  is  inevitable  where  dislocation  forward  takes  place,  for  in 
this  case  the  cornea  is  no  longer  moistened  by  the  tears ;  it 
becomes  dry  and  parchment-like,  and  presently  sloughs  leading 
to  destruction  of  the  eye. 

Many  cases  are  on  record  of  the  complete  loss  of  both  eyes  by 
this  means  :  Jessop  2  reports  three  such,  and  tabulates  twenty  - 
five  cases  from  the  literature  in  most  of  which  there  was  com- 
plete bilateral  loss  of  sight  from  corneal  lesions.  Griffith  3  reports 
the  case  of  a  woman  in  whom  both  eyes  were  destroyed  in  this 
way,  and  in  whom  after  the  removal  of  both  eyes,  the  lids  were 
still  unable  to  come  together  over  the  orbital  contents. 

The  proptosis  may  remain  in  greater  or  less  degree  for  years 
after  all  other  evidence  of  the  disease  has  disappeared.  Goulden 
has  operated  upon  a  woman  for  cataract  at  the  age  of  sixty- 
eight  in  whom  marked  proptosis  was  present,  the  residue  of  an 
attack  of  Graves'  disease  forty-nine  years  previously.* 

Different  explanations  of  the  immediate  cause  of  the  pro- 
ptosis have  been  advanced.  Thus  it  has  been  attributed  to 
vascular  engorgement,  to  contraction  of  unstriped  muscle 
tissue  in  the  orbit,  and  to  the  deposition  of  fat  in  the  orbit. 

It  is  evident  if  it  can  be  substantiated  that  the  proptosis 

1  Osier's  "System,"  1915,  iv.,  862. 

2  Trans.  Ophth.  Soc.  U.K.,  1896,  xvi.,  187. 

3  Ophth.  Review,  1898,  xvii.,  198. 

4  Foster  Moore,  Lancet,  1920,  701. 


EXOPHTHALMOS  175 

disappears  after  death,  or  that  it  may  come  on  in  a  night 
(Falta),  or  in  "  even  minutes  "  (Dock),  that  in  such  a  case  it  is 
not  due  to  an  increase  of  the  orbital  fat. 

Opportunities  of  really  testing  such  statements  are  necessarily 
few,  and  it  is  clear  that  the  accounts  of  patients  on  such  a 
point  are  of  little  or  no  value.  The  sudden  development  of 
Dalrymple's  sign  may  simulate  proptosis,  or  may  call  the 
attention  to  a  proptosis  which  previously  had  gone  unobserved. 
I  have  never  found  reason  to  believe  in  an  individual  case, 
that  proptosis  had  developed  suddenly,  nor  have  I  been  able 
to  procure  such  evidence  from  my  colleagues. 

Opportunities  for  post-mortem  examinations  are  rare  in  a 
general  hospital.  West 1  states  that  during  fourteen  years, 
only  three  patients  with  exophthalmic  goitre  died  in  St.  Bar- 
tholomew's Hospital. 

I  have  once  "only  had  an  opportunity  of  making  such  an 
examination,  and  in  this  woman  the  proptosis  after  death  was 
only  so  much  less  than  during  life,  as  might  be  expected  from 
the  draining  away  of  blood  from  the  orbit,  the  orbit  was  full 
of  fat,  and  no  other  abnormality  was  present.2 

I  have  in  addition  once  explored  an  orbit  during  life,  in  which 
proptosis  was  so  extreme,  that  the  lids  could  not,  even  under 
general  anaesthesia,  be  brought  together  over  the  cornea ;  there 
was  nothing  to  be  felt  but  soft  unresisting  fat,  which  together 
with  the  ocular  muscles  (to  be  referred  to  later)  seemed  to  be 
markedly  cedematous.3 

Bristowe  4  reports  four  autopsies  in  patients  dead  of  this 
disease.  In  three  of  them  he  states  :  "a  distinctly  increased 
quantity  of  yellow  fat,"  "  there  was  much  fat  in  the  orbits," 
"  both  orbits  contained  a  great  excess  of  fat,"  and  in  all  three 
no  other  abnormality  was  present.  It  is  worthy,  too,  of  note, 
that  whilst  the  orbits  were  full  of  fat,  three  of  the  bodies  are 
expressly  stated  as  being  emaciated.  In  the  fourth  case  it  is 
simply  stated  that  the  ocular  muscles  were  pale. 

1  Trans.  Ophth.  Soc.  U.K.,  1886,  vi.,  76. 

2  Foster  Moore.  Lancet,  1920,  701. 

3  Foster  Moore,  Pro.  Roy.  Soc.  Med.,  Oph.  and  Neurol.  Sect.,  March,  1921. 

4  Trans.  Ophth.  Soc.   U.K.,  1886,  vi.,  42  ;   "  Diseases  of  the  Nervous  System," 
1888,  141. 


176  GRAVES'   DISEASE 

In  a  case  of  Higgens,  reported  by  Goodhart,1  the  orbits 
showed  nothing  abnormal  ;  it  is,  of  course,  difficult  to  identify 
what  is  an  excess  of  fat  in  a  cavity  which  normally  is  full  of  it. 

Silcock,2  in  an  autopsy  to  be  referred  to  later,  in  which  all  the 
orbital  contents  were  examined  histologically,  says  :  "no  trace 
of  Muller's  musculus  orbitalis  was  visible." 

It  seems  unlikely,  a  priori,  that  this  single  manifestation  of 
so  sharply  defined  a  disease,  should  at  different  times  have 
three  entirely  and  widely  different  immediate  causes  for  its 
production.  It  is  evident  from  post-mortem  examination  and 
operative  experience,  that  at  any  rate  the  usual  cause  of  the 
prominence  of  the  eyes  is  an  increase  of  the  fat  in  the  orbit, 
and  this,  even  though  the  patient  is  greatly  emaciated. 

This  view  receives  additional  support  from  the  usual  slowness 
of  development  of  the  proptosis  and  from  its  long  persistence — 
forty-nine  years  (Goulden) — after  the  disease  has  ceased;  it 
may  also  be  that  oedema  of  the  orbital  tissues,  which  seemed 
undoubted  in  the  case  previously  referred  to,  and  which  is 
probably  responsible  for  the  bogginess  of  the  tissues  of  the  lids 
when  this  occurs,  is  an  additional  factor. 

As  has  been  stated,  the  condition  has  also  been  attributed  in 
certain  cases  to  engorgement  of  the  orbit  with  blood,  and  to  the 
contraction  of  unstriped  muscle  tissue  in  the  orbit  under 
sympathetic  control.  So  far  as  I  can  find  out,  the  only  support 
for  such  suggestions  is,  first,  the  statement  that  the  proptosis 
disappears  after  death,  secondly  that  it  may  develop  in  so 
short  a  time  that  the  possibility  of  the  deposition  of  fat  can 
be  ruled  out,  "  as  during  .the  night  "  or  "  even  in  a  few 
minutes,"  and  thirdly  that  in  some  cases  recession  of  the  eye 
is  reported  to  have  followed  division  or  resection  of  the 
sympathetic  nerve  in  the  neck. 

So  far  as  the  first  of  these  statements  is  concerned,  what  post- 
mortem evidence  I  have  been  able  to  find  is  in  direct  opposition 
to  it.  Of  the  second .  statement  I  can  only  say  that  I  have  not 
been  able  to  gather  from  my  own  experience  or  from  that  of 
my  colleagues  any  satisfactory  evidence  of  a  sudden  or  very 

1  Trans.  Path.  Soc.,  1874,  xxv.,  24(X 

z  Tram.  Ophth.  Soc.  U.K.,  1886,  vi.,  103. 


CAUSE  OF  EXOPHTHALMOS  177 

rapid  onset,  and  I  suggest  that  in  those  instances  in  which  it  has 
seemed  to  occur,  it  may  have  been  due  to  the  sudden  develop- 
ment of  Dalrymple's  sign  simulating  proptosis,  or  unmasking 
an  early  proptosis  which  up  to  then  had  been  overlooked.  In 
this  connection  it  is  of  some  interest  to  observe,  that  in  the 
contrary  case  it  is  not  rare  for  patients  who  have  developed  a 
partial  ptosis  from  third  nerve  involvement,  to  come  complain- 
ing that  the  eye  is  getting  smaller  or  is  sinking  into  the  head. 

In  addition  I  know  of  no  anatomical  evidence  of  the  existence 
of  such  unstriped  muscle  tissue  in  the  orbit  in  man  as  could 
produce  the  effect,  and  Silcock's  histological  examination 
already  referred  to,  failed  to  reveal  any  trace  of  Miiller's  muscle. 

With  regard  to  the  effects  of  division  or  resection  of  the 
sympathetic  nerve  in  the  neck,  there  is  no  unanimity  of  opinion 
as  to  its  effect  on  the  exophthalmos.  Thus  Jaboulay  x  says  of 
eight  cases  in  which  the  sympathetic  was  divided,  that  the 
exophthalmos  was  favourably  influenced  in  all  cases  after  the 
first  day,  a  view  which  receives  some  support  from  Gayet.2 

Balacescu,3  on  the  other  hand,  finds  little  effect  on  the 
exophthalmos  produced  by  section  of  the  nerve,  a  view  which 
coincides  with  that  of  Pean.4 

If  the  exophthalmos  is,  indeed,  due  to  contraction  of  unstriped 
muscle  tissue  under  the  control  of  the  sympathetic  nerve,  it 
would  be  expected  that  the  effect  of  division  would  be  imme- 
diate, and  of  such  a  degree  that  there  could  be  no  room  for 
views  so  diametrically  opposed  as  to  its  effect. 

I  believe  it  may  be  said  that  the  immediate  cause  of  the 
exophthalmos  of  Grave's  disease  is  an  increase  of  the  orbital 
fat,  perhaps  combined  with  oedema  of  the  tissues,  and  that 
there  is  at  present  no  satisfactory  evidence  of  any  other  cause 
of  it. 

Defective  Ocular  Movements. — Paralysis  of  some  of  the 
external  ocular  muscles  is  at  times  seen,  but  ophthalmoplegia 

c^^ 
interna  does  not  occur. 

The  external  rectus  is  most  often  affected,  but  at  times  the 

1  Chir.  du  Grand  Sympath.  ct  du  Corps  Thyroide,  1900,  79. 

2  Lyon  Medical,  1896,  Ixxxii.,  423. 

3  Archiv.f.  Klinische.  Chir.,  1902,  Ixvii.,  59. 

4  Bull.  Acad.  de  Med.,  1897,  xxxviii.,  132. 

12 


178  GRAVES'   DISEASE 

internal  rectus  is  involved,  and  it  is  stated  that  occasionally 
all  the  external  muscles  may  be  paralysed  or  defective  in 
action  ;  the  effect  may  be  transient  or  intermittent. 

Cases  are  reported  by  Lang  and  Pringle,1  West,2  Ballet,3 
Bristowe,4  Warner,5  Maude,6  Voss,7  and  others,  in  some  of 
which  other  cranial  nerves  have  also  been  involved. 

It  is  probable  that  the  defective  movements  are  due  to 
changes  in  the  muscles,  rather  than  in  their  nerves  of  supply,  a 
view  which  is  supported  by  the  distribution,  and  by  the  fact 
that  definite  changes  have  been  found  in  the  muscles. 

In  a  case  of  my  own,  the  recti  muscles  during  life,  instead  of 
being  thin,  flat  bands,  were  fusiform  bellies,  almost  as  thick  as 
the  top  of  one's  little  finger,  due,  it  seemed  to  me  at  the  time,  to 
cedematous  swelling,  but  perhaps  due  to  fatty  infiltration 
(vide  infra). 

In  a  case  of  Bristowe's  8  in  which  ophthalmoplegia  externa 
had  occurred  and  which  came  to  autopsy,  the  muscles  are 
described  as  being  pale. 

A  most  careful  examination  of  a  case  was  made  by  Silcock,9 
all  the  orbital  tissues  being  examined  histologically.  He  says  : 
"  There  was  nothing  abnormal  in  the  orbits  except  a  most 
noticeable  yellowish,  patchy  discoloration  of  the  recti  and 
oblique  muscles,  which  a  microscopic  examination  proved  to 
be  due  to  interfascicular  fatty  infiltration,  the  muscle  fibres 
themselves,  though  somewhat  pale,  showing  no  trace  of  fatty 
degeneration.  The  levatores  palpebrarum  markedly  con- 
trasted with  the  other  extrinsic  ocular  muscles  inasmuch  as 
they  were  normal  in  colour,  or  perhaps  merely  a  shade  paler 
than  normal." 

The  vessels  and  nerves  were  all  examined  histologically,  and 
showed  no  abnormality. 

1  Trans.  Ophth.  Soc.  U.K.,  1886,  vi.,  105. 

2  Trans.  Ophth.  Soc.  U.K.,  1886,  vi.,  79. 
s  Recueild'Ophthal.,  1888,  321. 

*  Brain,  1886,  viii.,  313. 

5  Med.  Times  and  Gaz,  1882,  ii.,  541. 

6  St.  Bartholomew's  Hosp.  Reports,  1891,  xxvii.,  133. 

7  Deutsche  med.  Wochenschr.,  1903,  xxix.,  590. 

"  Diseases  of  the  Nervous  System,"  1888,  141. 
9  Trans.  Ophth.  Soc.  U.K.,  1886,  vi.,  43. 


•Macutar 


Dorsal  Grossed  Pascicului 
Ventral        " 
Dor  sal  Uncro  s  scd 
Ventral 


|          [  Macular 

FIG.  59. — Diagram  based  on  Henschen's  conception  of  the  disposition  in 
the  optic  nerve  of  the  several  fasciculi.  There  is  indicated  an  hypo- 
thetical involvement  of  the  ventral  crossed  fasciculi  from  the  right 
eye,  which  has  produced  Stage  I.  (see  Fig.  60)  of  a  right  temporal 
defect  (c/.  included  perimetric  field).  The  path  of  the  fibres  between 
the  point  of  pressure  at  the  chiasm  and  the  retina  is  indicated  by 
the  short  arrows.  (Harvey  Gushing  and  Clifford  B.  Walker.) 


Stage  1 


Stage  III 


StageVl 
V-34 


Fio.  60. — Showing  the  eight  stages  of  a  progressing  right  temporal  field 
defect  in  pituitary  disease.  (Harvey  Gushing  and  Clifford  B. 
Walker.) 


PITUITARY  DISEASE  179 

It  seems  probable,  that  when  deficient  movement  of  the 
eyes  occurs  in  Graves'  disease,  it  is  due  to  impairment  of 
function  of  the  muscles  as  a  result  of  their  infiltration  by  fat 
or  by  oedema,  or  perhaps  by  both,  and  that  marked  exoph- 
thalmos,  when  present,  will  introduce  a  mechanical  factor 
which  will  contribute  towards  the  same  effect. 

I  have  once  seen  a  typical  retro-bulbar  neuritis  associated 
with  Graves'  disease  which  recovered  completely  in  the  course 
of  about  three  months,  but  whether  this  was  a  part  of  the 
disease  or  an  accidental  complication  of  it  I  am  unable  to  say. 

PITUITARY   DISEASES. 

Owing  to  the  proximity  of  the  pituitary  body  to  the  optic 
pathways,  ocular  lesions  constitute  some  of  the  most  important 
complications  of  pituitary  disease,  and  almost-all  of  these 
complications  are  dependent  upon  the  direct  pressure  of  the 
enlarged  gland. 

For  much  of  our  recent  and  improved  knowledge,  and 
consequent  earlier  recognition  of  the  diseases  of  the  pituitary, 
we  are  dependent  upon  the  skill  and  labour  of  Harvey  Gushing 
and  C.  B.  Walker,  from  whose  work  much  of  what  follows  is 
taken. 

The  chief  changes  to  be  considered  are  defects  of  the  visual 
fields  and  objective  signs  in  the  eye  grounds. 

There  are  three  separable  factors  in  pituitary  disorders  1  :— 

(1)  Neighbourhood  signs  and  symptoms  shown  by  evidence 

of  pressure  on  adjoining  structures,  chiasm,  cranial 
nerves,  uncinate  gyrus,  crura  cerebri,  and  sella  turcica. 

(2)  General  pressure  symptoms,  in  case  the  growth  becomes 

large  enough  to  cause  considerable  cerebral  deformation 
or  to  obstruct  the  cerebro -spinal  fluid  circulation  by 
blocking  the  foramen  of  Munro. 

(3)  Constitutional    or   glandular    symptoms,    either    in    the 

role  of  over-  or  under-hypophysical  activity. 

With  the  last  of  these  we  shall  not  find  ourselves  concerned. 

1  Gushing  and  Walker,  Archiv.  ofOphth.,  1916,  xlv.,  407,  and  1918,  xlvii.,  119  ; 
Brain,  1914,  xxxvii.,  341. 

12—3 


180 


PITUITARY  DISEASE 


Defects  in  the  Fields  of  Vision. — These  have  been  studied 
with  minute  care  in  all  their  phases  of  deterioration  and  subse- 
quent recovery  by  Walker,  by  whom,  for  purposes  of  description, 
they  are  divided  into  four  groups  :— 

(1)  Bitemporal  hemianopia. 

(2)  Homonymous  hemianopia. 

(3)  Blindness  in  one  or  both  eyes. 

(4)  Irregular  defects. 


FIGS.  61  and  62. — To  show  the  progress  of  the  loss  of  the  upper  temporal 
fields  of  vision  in  a  lad  of  seventeen  suffering  from  a  pituitary  tumour. 
The  dotted  line  shows  the  field  taken  forty-two  days  before  the  con- 
tinuous line. 

With  regard  to  these  defects  it  is  strongly  emphasised  that 
the  process  rarely  advances  with  equal  steps  in  the  two  eyes, 
and  consequently  it  is  unusual  to  find  bilaterally  symmetrical 
defects  ;  and,  further,  it  is  important  to  realise  that  there  are 
probably  recessions  and  advances  from  time  to  time,  though 
the  general  tendency  is  towards  advance. 

/     Of  271  cases  of  pituitary  lesions  148  showed  some  abnormality 
of  the  visual  fields.     Of  these  forty-seven  were  bitemporal 


VISUAL   FIELDS 


181 


defects,  twenty-two  were  homonymous,  and  seventy-nine  had 
one  or  both  eyes  blind,  so  it  could  not  be  stated  what  form  of 
defect  was  present. 

Amongst  seventy -nine  cases  reported  by  de  Lapersonne  l 
bitemporal  defects  were  present  in  32  per  cent,  whilst  amongst 
UhthofFs  cases,2  of  119  without  signs  of  acromegaly  or  other 
trophic  disturbance,  28  per  cent,  had  bitemporal  defects, 


FIG.  62 

whilst  of  207  cases  with  acromegaly,  etc.,  42  per  cent,  had  such 
defects  in  the  fields  of  vision. 

The  most  typicaTform  of  field  is  a  bitemporaTnemianopia  ; 
but,  contrary  to  what  might  be  expected,  a  hemianopia  of 
homonymous  type  is  by  no  means  rare.  According  to  Uhthoff 
it  is  about  ten  times  as  uncommon  as  a  bitemporal  defect,  but 
Gushing  and  Walker's  figures  indicate  that  it  is  only  about 
twice  as  infrequent. 

Besides^tliese  defects  which  can  be  classified,  there  is  left 
a  large  group  of  cases  in  which,  owing  to  the  advanced  condition 

1  Quoted  by  Werner,  Trans.  Ophth.  Soc.  U.K.,  1912,  xxxii.,  295. 

2  Bowman  Lecture,  Tram.  Ophth.  Soc.  U.K.,  1914,  xxxiv.,  xcii. 


182 


PITUITARY  DISEASE 


of  the  visual  loss  in  one  or  both  eyes  at  the  time  they  first 
come  under  observation,  it  is  not  possible  to  say  what  was 
originally  the  condition. 

It  is  suggested  by  de  Schweinitz  and  Carpenter  l  that 
probably  many  individual  variations  in  decussation  occur, 
from  perhaps  entire  absence  of  crossing  to  total  decussation  ; 
any  such  variations  would  influence  the  type  of  field  defect  in 
the  cases  und^consideration. 

In  a  majority  of  cases,  whether  intra-  or  supra -sellar,  the  first 


(i  \\ 
C.43.WT 

FIG.  63 

FIGS.  63  and  64  are  charts  of  the  visual  fields  of  a  man  aged  thirty-two 
suffering  from  an  hypophysial  struma  with  optic  atrophy.  He  had 
had  occasional  diplopia  and  trouble  with  his  eyes  for  six  or  seven 
years.  Fig.  63  shows  the  fields  before  operation.  Both  eyes  are 
just  short  of  Stage  IV.  (see  Fig.  60).  Note  the  island  of  vision  in  the 
.right  temporal  field. 

perimetric  indication  of  the  process  is  shown  by  a  slant  in  the 
boundary  of  the  upper  temporal  form  field,  and  a  corresponding 
quadrantal  defect  in  the  colour  periphery  (Fig.  60,  Stage  I.). 

In  alhca^es  the  loss  of  colour  perception  is  in  advance  of  that 
for  form.  The  defect  commences  in  the  upper  temporal  field, 
and  progresses  more  rapidly  in  the  centre  than  towards  the 
periphery,  so  that  a  gourd-shaped  field  is  produced  (Fig.  60, 
Stage  III.),  and  it  comes  about  as  a  result  of  the  further 
advance  of  this  same  process,  that  an  island  01  field  may 

1  Jour.  Amer.  Med.  Asaoc.,  1905,  xliv.,  81. 


PHYSIOLOGICAL  BLOCK 


183 




become  cut  off  in  the  lower  temporal  part,  whilst  for  the  rest     ,, 
the  process  results  in  a  sharp-cut  temporal  hemianopia,  the  ^V/ 
nasal  field  remaining.      This   temporal  island  of  field  is  very 
constantly  about  50  degrees  from   the   fixation    point   on  or 
below  the  horizontal  mRridla.ii^(Figs_6^and  64). 

The  nasal  field  next  begins  to  shrink  approximately  con- 
centrically, so  that  ultimately  only  a  small  functioning  patch 
remains  in  the  lower  nasal  quadrant  (Fig.  60,  Stage  VII.). 


FIG.  64. — From  the  same  patient  as  Fig.  63,  taken  twenty -four  days 
later  and  eighteen  days  after  operation.  Shows  increase  of  the 
visual  fields  with  recession  towards  Stage  III.  (Fig.  60)  and  en- 
gulfing of  the  island  in  the  right  temporal  field  by  the  16  sq.  cm. 
disc.  Notice  the  development  of  a  symmetrical  island  in  the  left 
temporal  field.  (Gushing  and  Walker.) 

'  The  gradual  evolution  of  these  half  field  defects  contrasts 
sharply  with  the  common  cortical  hemianopias  which  are  usually 
of  sudden  onset  and  complete  from  the  first. 

In  cases  of  supra-sellar  tumours  in  the  interpeduncular  space, 
contrary  to  what  might  be  expected,  the  field  defects  do  not 
appear  first  in  the  lower  temporal  field,  but  frequently  follow 
the  typical  stages. 

Physiological  Block. — Great  importance  is  attached  to  the 
evidence  supplied  by  these  cases  as  showing  that  loss  of  visual 
field  and  even  marked  pallor  of  the  optic  disc  is  not  at  first  due 
to  organic  change  in  the  nerve,  but  is  produced  by  what  Gushing 


184  PITUITARY  DISEASE 

calls  a  physiological  block,  and  consequently  the  prospect  of  re- 
covery of  function  if  pressure  can  be  relieved  is  much  improved. 
/  Gushing  states  that  vision  has  been  restored  to  blind  eyes 
even  when  the  pupil  reflex  had  been  lost,  and  that  in  several 
cases  where  the  ophthalmoscope  had  shown  advanced  atrophy 
and  the  perimeter  advanced  defect,  only  a  scattered  degenera- 
tion was  found  histologically. 

Forti 1  makes  the  interesting  observation  that  in  pregnant 
women  in  perfect  health  there  is  a  restriction  of  the  temporal 
visual  fields  ;  rt  is, presumably  due  to  hypertrophy  of  the 
gland  during  pregnancy,  and  is  afterwards  recovered  from. 

The  occurrence  of  scotom&ta  in  the  visual  field  is  not  rare. 
They  were  first  accurately  investigated  by  de  Schweinitz  and 
Holloway  2  who  showed  that  they  are  usually  either  macular 
or  paramacular,  probably  because  the  corresponding  fibres  are 
more  vulnerable  than  those  from  the  peripheral  retina,,  and  it 
is  perhaps  for  this  same  reason  that  they  tend  tcTpersist  after 
the  rest  of  the  field  of  vision  is  restored. 

Mode  of  Restoration  of  the  Visual  Fields. — When  relief  of 
pressure  has  been  afforded  by  operative  measures,  the  recession 
of  the  defects  takes  place  in  a  sequence  the  reverse  of  that 
characterising  the  stage  of  the  advance,  but  as  already  stated, 
scotomata,  if  present,  tend  to  persist  (Figs.  63  and  64). 

The  restoration  of  the  visual  field  peripheries  is  possible  even 
when  the  process  has  advanced  beyond  the  stage  of  half 
vision,  and  some  sight  may  be  regained  even  after  blindness 
has  occurred,  provided  that  it  has  not  been  of  too  long 
duration. 

The  recovery  of  the  affected  parts  of  the  field  may  be  very 
rapid  after  operation,  and  nearly  all  cases  improve  if  operated 
on  before  the  stage  of  complete  loss  of  temporal  field,  the  nasal 
fields  being  intact.) 

Fisher  3  suggests  that  the  distortion  of  the  fields  of  vision 
is  the  result  of  traction  upon  the  nerve  and  chiasm  rather  than 
of  direct  pressure  upon  these  parts,  and  that  the  vulnerability 

1  Archiv.  Di  Oltalmol.,  xvii.,  8. 

2  Jour.  Amer.  Med.  Assoc.,  1912,  lix.,  1041. 

1  Proceed.  Roy.  Soc.  Med.,  London  Sect.  Neurol.  &  Ophth.,  1912-13,  vi.,  3,  Ixvii. 


RESTORATION  OF  FIELDS  185 

of  the  macular  fibres  is  associated  with  their  highly  specialised 
function. 

Walker  and  Gushing  believe  that  pressure  and  traction  go 
hand  in  hand,  and  from  their  post-mortem  examinations  they 
consider  that  the  tracts  suffer  more  than  the  nerves  or  chiasm, 
for  they  may  be  thinned  out  into  mere  ribbons  even  when  the 
chiasma  shows  little  deformity  ;  their  post-mortem  examina- 
tions also  demonstrate  that  the  chiasm  may  undergo  con- 
siderable distortion  without  producing  demonstrable  alterations 
in  the  visual  fields,  for  in  about  20  per  cent,  of  cases  actually 
showing  neighbourhood  signs  there  was  no  perimetric  change. 

Atrophy  in  the  optic  tracts  considerably  antedates  that  in 
the  optic  nerves,  where  the  fibres  may  be  preserved  by  their 
retinal  ganglion  cells  for  some  years  after  complete  functional 
blindness  has  occurred. 

Gushing  and  Walker  x  state  that  in  20  per  cent,  of  patients 
showing  pronounced  neighbourhood  symptoms  there  is  no 
demonstrable  perimetric  change. 

It  will  be  gathered  that  the  chiasmal  cross-roads  are  capable 
of  considerable  distortion  by  a  growth  in  the  interpeduncular 
space  without  the  fields  of  vision  being  encroached  upon. 

They  also  show  that  cases  in  which  normal  fields  were  plotted 
shortly  before  death,  have  exhibited  an  extraordinary  degree 
of  deformation  and  elongation  of  the  chiasma  on  post-mortem 
examination.  They  conclude,  therefore,  that  an  existent, 
clear-cut  bi-temporal  hemianopia  is  an  evidence  of  a  medial 
lesion  most  often  due  to  a  tumefaction  of  the  hypophysis  or 
its  stalk,  but  that  the  reverse  argument  does  not  hold,  that  the 
absence  of  this  characteristic  defect,  or,  indeed,  of  any  defect 
whatsoever  in  the  fields,  necessarily  speaks  against  a  primary 
pituitary  or  interpeduncular  growth. 

Of  eighty -one  cases  of  pituitary  disease  exhibiting  field 
defects  fourteen  were  acfoinegalics. 

Ophthalmoscopic  Changes. — The  most  common  ophthalmo- 
scopic  abnormality  is  a  varying  degree  of  optic  atrophy,  and 
this  is  of  a  primary  rather  than  a  consecutive  type. 

1  Brain,  1914,  xxxvii.,  341. 


186  PITUITARY  DISEASE 

It  is  produced  by  direct  pressure  upon  the  nerve,  and  by  this 
means  the  subarachnoid  space  of  the  nerve  sheath  is  cut  off 
from  the  general  cerebral  subarachnoid  space,  and  so  is  pro- 
tected against  an  increase  of  intra-cranial  pressure  should  such 
occur,  with  the  result  that  papillcedema  is  of  unusual  occurrence 
(see  p.  16). 

Gushing  and  Walker  say  in  a  few  cases  "  a  low  grade  of 
oedema,  occasionally  with  new  tissue  formation,  has  been 


FIG.  65. — To  show  the  decussation  of  the  optic  nerve  fibres  at  the  chiasmn, 
from  a  case  in  which  the  left  eye  had  been  removed  some  years  pre- 
viously. (Gordon  Holmes.) 

superimposed  on  a  primary  atrophy,  when  intra-cranial  pressure 
has  become  extreme  and  the  nerve  sheaths  have  not  been 
occluded  by  the  growth." 

Uhthoff  finds  active  changes  in  the  nerve  head  to  be  of  more 
common  occurrence  ;  thus  he  finds  either  "  choked  disc  "  or 
optic  neuritis  to  be  present  in  24  per  cent,  of  cases  without 
acromegaly  or  trophic  disturbances,  dystrophia  adiposo- 
genitalis,  etc.,  and  in  11  per  cent,  of  cases  with  such  distur- 
bances. 


INFANTILE  SCURVY  187 

Fisher  1  looks  upon  primary  optic  atrophy,  and  not  papill- 
cedema,  as  the  feature  of  the  disease. 

Exophthalmos  of  a  low  degree  is  not  infrequent  ;  amongst 
ninety-one  cases  Strzeminski  2  found  it  present  in  seventeen, 
but  believes  it  is  more  common  than  this.  Gushing  states  that 
some  degree  of  exophthalmos  has  been  shown  by  almost  all 
of  the  patients  with  tumour  ;  it  is  rare  in  the  absence  of  a 
definite  growth.  He  looks  upon  it  as  due  to  obstruction  of  the 
flow  in  the  cavernous  sinus/* 

Affections  of  the  ocular  nerves  are  of  common  occurrence, 
and  of  them  the  third  is  most  often  affected  ;  the  sixth  is  much 
less  often  involved.  In  Uhthoff's  cases  the  third  was  affected 
four  times  as  frequently  as  the  sixth.  Hertel  3  says  the  sixth 
nerve  is  very  rarely  affected,  but  the  third  is  frequently. 

This  preponderance  of  the  involvement  of  the  third  nerve 
over  the  sixth  is  the  more  significant  from  the  high  vulnerability 
of  the  latter  in  all  sorts  of  intra -cranial  lesions. 

Nystagmus  occurred  in  about  4  per  cent,  of  Uhthoff's  cases. 

INFANTILE   SCURVY   (BARLOW'S   DISEASE). 

In  this  disease,  at  a  time  when  spongy  bleeding  gums, 
hsematuria,  subperiosteal  haemorrhages,  and  other  symptoms 
occur,  bleeding  may  also  take  place  under  the  periosteum  or  into 
the  tissues  of  one  or  both  orbits. 

It  is  usually  a  late  symptom,  and  Barlow  has  reported  a  case 
in  which  it  was  the  only  symptom  of  the  disease.  A  committee 
of  the  American  Pediatric  Society,  as  a  result  of  the  investi- 
gation of  379  cases  of  the  disease,  found  proptosis  to  be  present 
in  forty  Instances.4 

It  usually  comes  on  suddenly,  the  eye  being  pushed  forwards 
and  somewhat  down  and  outwards,  the  common  direction  for 
an  uncontrolled  proptosis.  The  fit  of  crying  by  which  its 
development  is  accompanied  is  effect  rather  than  the  cause. 

1  Trans.  Ophth.  Soc.  U.K.,  1911,  xxxi.,  91. 

2  Archiv.  d'ophlhal,  1897,  xvii.,  108. 

3  v.  Graefe's  Archiv.  f.  Ophth.,  1895,  xli.,  187. 

*  Boston  Med.  and  Surg.  Jour.,  1898,  cxxxviii.,  605. 


188  KERATO-MALACIA 

If  the  blood  infiltration  transgresses  the  orbital  fascia, 
ecchymosis  of  the  lids  occurs  ;  it  may  take  the  form  of  a  purple 
line  along  the  orbital  margin  or  a  bulky  infiltration  of  the 
tissues.1  When  bleeding  takes  place  from  any  cause  into  so 
poorly  vitalised  a  tissue  as  fat  it  is  slow  in  absorbing,  and 
Holmes  Spicer  2  has  shown  that  the  prominence  of  the  eyes  in 
these  cases  takes  long  to  disappear.  He  quotes  a  case  in  which, 
after  seven  months,  one  eye  was  still  abnormally  prominent. 

Cases  of  unilateral  proptosis  in  healthy  new-born  infants  have 
been  reported  by  Borland  3  and  Crawford,4  due,  apparently,  to 
haemorrhage  into  the  orbit  during  birth,  and  followed  by 
complete  recovery. 

KERATO-MALACIA. 

This  is  a  distinctive  disease  of  the  cornea  which  usually 
occurs  in  infants  under  one  year  of  age  who  are  seriously  ill  as  a 
result  of  improper  feeding. 

The  subjects  of  the  disease  have  almost  invariably  been  fed 
on  starchy  foods  with  an  insufficiency  of  fresh  milk,  and  have 
a  wizened,  dusky  appearance  ;  in  fourteen  out  of  thirty-one 
cases  there  was  a  fatal  termination.5 

First  one  eye  then  the  other  is  involved  ;  the  lid  margins  are 
covered  with  sticky  shreds  of  meibomian  secretion  ;  the  con- 
junctiva becomes  dry,  wrinkled,  and  greasy -looking,  and  is  not 
moistened  by  the  tears,  and  foam-like  patches  are  developed 
on  it.  The  cornea  is  dry,  lustreless,  and  cloudy,  and  ultimately 
becomes  entirely  disintegrated.  A  striking  feature  is  the  almost 
entire  absence  of  signs  of  inflammation  throughout  the  course 
of  the  disease.6 

At  one  time  I  examined  the  eyes  of  a  considerable  number 
of  rats  which  were  being  experimented  on  by  Professor  Gowland 
Hopkins  by  being  fed  upon  a  diet  which  was  free  of  the  fat 
soluble  vitamine.  Many  of  them  developed  a  condition  of  the 

1  Stephenson,  Proc.  Roy.  Soc.  Med.,  London,  1914-15.  Section  Dis.   of  Child., 
viii.,  44-47. 

2  Trans.  Ophth.  Soc.  U.K.,  1892,  xii.,  36. 

3  Lancet,  1904,  ii.,  1344. 

4  Lancet,  1904,  ii.,  1450. 

*  Kapuscinski,  v.  Graefe's  Archiv.f.  Ophth.,  1912,  Ixxxii.,  229. 

•  Holmes  Spicer,  Trans.  Ophth.  Soc.  U.K.,  1893.,  xiii,  45. 


EXPERIMENTAL    WORK  189 

cornea  which  appeared  to  be  similar  to  kerato-malacia  as  it 
occurs  in  children.  The  condition  has  recently  been  carefully 
investigated  by  Stephenson  and  Clarke  l  and  McCollum  and 
Simonds.2 

The  former  investigators  conclude  that  the  condition  directly 
attributable  to  dietetic  deficiency  is  a  predisposition  to 
bacterial  infection  of  the  cornea,  leading  to  purulent  symptoms 
and  destruction  of  tissue.  They  show  that  the  eye  disease  is 
by  no  means  a  constant  precursor  of  death  in  these  rats,  but 
that  in  every  case  of  corneal  disease  in  which  it  was  attempted  a 
cure  was  effected  by  the  replacement  of  the  fat  soluble  factor. 
The  (latter  investigators,  also  working  with  rats,  definitely , 
associated  xerophthalmia  with  the  absence  from  the  diet  of  fat" 
soluble  vitaminey'  They  say  :  "  There  are,  then,  two  deficiency 
diseases  in  the  sense  in  which  Funk  employed  this  term.  One 
of  them  is  polyneiiritis — the  other,  we  believe,  is  the  syndrome 
described  above,  in  which  the  two  most  prominent  features  are 
emaciation  and  xerophthalmia." 

Holmes  Spicer  has  seen  a  similar  condition  of  the  cornea  in 
a  dog  fed  exclusively  on  biscuit,  in  which  a  meat  diet  produced 
an  extraordinarily  rapid  cure. 

1  Biochem  Jour.,  1920,  xiv.,  502. 

2  Jour.  Biol.  Chem.,  1917,  xxxii,  181  and  347 

i^~~*£^&* 


CHAPTER  V 

Syphilis — Gross  tuberculous  lesions — Miliary  tuberculosis— Corebro- 
spinal  meningitis — Pyaemia  and  septicaemia — Infective  endocarditis 
— Facial  erysipelas — Gonococcal  eye  affections — Eye  affections  in 
dysentery — Tetanus — Diphtheria — Leprosy. 

SYPHILIS. 

LESIONS  affecting  the  eyes  or  their  external  musculature  are 
common  in  both  the  congenital  and  the  acquired  disease  ,  they 
may  occur  as  a  manifestation  of  intra -cranial  syphilitic  disease 
or  as  a  local  manifestation  of  general  systemic  infection. 

A  primary  syphilitic  chancre  occasionally  occurs  on  the 
conjunctiva,  especially  on  that  of  the  lower  lid.  It  is  followed 

bv  the  usual  constitutional  manifestations. 

j  ^   ~  \ - 

The  chancre  is  generally  larger  and  is  less  indurated  than  is 
usual  in  genital  chancres,  the  lymphatic  glands,  especially  the 
pre-auricular  and  submaxillary,  are  much  enlarged  ,  they  are 
not  so  firm  and  shotty  as  are  those  in  the  groin  in  genital 
chancres. 

There  is  some  evidence  l  to  show  that,  should  the  patient 
develop  interstitial  keratitis — a  rare  disease  in  acquired  syphilis, 
and.  unlike  its  counterpart  in  congenital  syphilis,  often  uni- 
lateral 2 — it  is  more  likely  to  develop  in  the  eye  which  has 
been  the  site  of  a  primary  chancre  than  in  the  opposite  eye. 

I  have  once  seen  the  lachrymaTsac  the  site  of  a  primary 
chancre,  it  was  as  if  it  had  been  injected  with  plaster  of 
Paris  ;  there  was  no  ulceration  or  excoriation  or  any  abnor- 
mality to  be  seen  at  the  punctum  or  inner  canthns  ;  all  the 
usual  constitutional  signs  followed,  and  cicatrisation  was 
followed  by  troublesome  epiphora.  Previously  there  had  been 
no  trouble  of  any  sort  with  the  eye,  and  it  seems  all  but  certain 
in  this  case  that  the  spirochsete  obtained  entrance  to  the  body 
through  an  intact  mucous  surface. 

1  Treachcr  Collins,  Roy.Lond.  Ophth.  Hosp.  (Moor field-?)  Reports,  1906,  xvi.,  16. 
9  Fisher,  Trans.  Ophth.  Soc.  U.K.,  1908,  xxviii.,  59. 


MORTALITY  IN   THE  THIRD   GENERATION     191 

INTERSTITIAL   KERATITIS. 

There  are  a  number  of  diseases  of  the  cornea  to  which  the 
name  interstitial  keratitis,  used  in  its  anatomical  sense,  is 
applicable  ;  the  term,  however,  has  come  to  be  applied,  without 
qualification,  to  a  disease  which  is  a  very  sharply  denned  entity 
as  regards  incidence,  morbid  appearances,  and  clinical  course, 
and  for  this  disease  there  is,  I  believe,  a  single-ctmse,  and  that 
is  syphilis  ;  and  whilst  it  very  occasionally  arises  after  acquired 
infection,  it  is  almost  invariably  due  to  the  congenital  disease. 

It  is  a  disease  of  the  young,  and  the  years  between  five 
and  seventeen  will  include  a  large  majority  of  the  cases.  I 
have,  however,  seen  it  develop  in  its  typical  form  in  a  woman 
of  thirty-nine  who  had  Hutchinson's  teeth  and  was  deaf,  and 
occasionally  an  infant  is  born  with  the' disease  established. 
Of  the  sixty-four  cases  which  were  so  carefully  investigated 
by  Hutchinson  l  nine  were  under  the  age  of  five,  and  the  sex 
incidence  showed  a  marked  preponderance  of  females  in  the 
proportion  of  1'8  to  1.  It  is  very  common  to  find  other  undeni- 
able signs  of  the  congenital  taint,  e.g.,  a  characteristic  facies, 
scars,  deafness,  Hutchinson's  teeth,  etc.  ;  and  if  the  child  be 
stripped,  synovitis  of  the  knees,  tibial  nodes  or  curved  tibiae 
are  not  infrequently  found,  though  both  the  patient  and  the 
mother  may  be  ignorant  of  their  existence. 

On  the  other  hand,  it  is  not  very  rare  to  find  no  clinical 

evidence  of  the  congenital  disease  other  than  the  eye  condition. 

Hutchinson's    teeth  are  by  no  means   always  present,  but  I 

/believe  it  to  be  true  that  a  patient  in  whom  they  are  present 

will,  sooner  or  later,  certainly  suffer  from  interstitial  keratitis. 

It  is  of  interest  here  to  refer  to  the  mortality  amongst  the 
children  of  those  who  are  the  subject  of  congenital  syphilis 
as  evidenced  by  the  presence  of  interstitial  keratitis.  Hutchin- 
son found  that  fifty -three  such  mothers  bore,  between  them, 
371  children,  and  that  of  this  number  179  only  survived. 
He  states  :  "  There  can  be  little  doubt,  despite  the  many 
fallacies  to  which  statistics  expose  us,  that  this  rate  of  mortality 
is  very  high."  Treacher  Collins  2  found  that  twelve  such  women 

1  Roy.  Lond.  Ophth.  Hasp.  Rep.,  1857-9,  i.,  191,  226  ;  1859-60,  ii.,  54. 
-  Roy.  London  Ophth.  Hasp.  Reports,  1903,  xv.,  208. 


192  INTERSTITIAL  KERATITIS 

had  thirty-four  living  children,  of  whom  twenty-five  were  pre- 
sumably healthy  and  fourteen  were  certainly  so.  It  is  clear, 
therefore,  that  women  the  subjects  of  congenital  syphilis  may 
certainly  have  healthy  children,  free  from  all  taint  of  the 
disease.  He  states  :  "  The  difference  between  the  average 
infant  mortality  in  London  (16  per  cent.)  and  amongst  the 
grandchildren  of  those  who  have  had  primary  syphilis  (36-6 
per  cent.)  remains  very  marked,  so  as  to  make  it  impossible 
to  avoid  the  inference  of  the  presence  of  some  dystrophic 
influence." 

It  has  been  shown  by  Harman,1  who  examined  the  knee-jerks 
of  100  normal  children  and  100  children  who  had  had  inter- 
stitial keratitis,  that  there  was  no  deficiency  in  the  knee-jerks 
of  the  latter  group. 

In  Acquired  Syphilis. — Interstitial  keratitis  does,  in  a  few 
cases,  result  from  acquired  syphilis. 

The  interval  between  infection  and  the  development  of  the 
corneal  disease  may  vary  from  four-and-a-half  months  2  to 
twenty  yearsj 3  36  per  cent,  of  cases  occur  in  the  second 
year,4  and  whereas  in  the  congenital  disease  both  eyes  are 
always  involved,  the  disease  is  frequently  unilateral  where  the 
infection  is  acquired.5 

Iritis. — This  occurs  in  small  infants  in  congenital  syphilis, 
or  soon  after  infection  in  the  acquired  disease. 

The  variety  occurring  in  congenital  cases  was  studied  in 
twenty-three  cases  by  Johnathan  Hutchinson.6  He  showed 
that  it  was  much  more  common  in  female  than  in  male  children  ; 
that  the  average  age  of  onset  was  five-and7a:Ealf  months,  the 
oldest  of  his  twenty-three  cases  being  sixteen  months  ;  that  in 
about  one-half  the  cases  it  was  unilateral  ;  that  the  signs  of 
inflammation  are  slight,  but  the  effusion  of  lymph  is  usually 
free.  It  may  be  said  that/if  a  small  infant  who  shows  no  signs 

1  Opfith.  Review,  1903,  xxii.,  241. 

2  Lawford,  Trans.  Ophth.  Soc.  U.K.,  1900,  xx,  68. 

3  Terson,  Recueil  d'Ophthal.,  1899,  23. 

4  Verhaeghe,  Gazette  deshdpit.,  1904,  Ixxvii,  1141. 

&  Fisher,  Trans.  Ophth.  Soc.  U.K.,  1908,  xxviii,  66 ;  Carpenter,  Trans,  Arner. 
Ophth  Soc.,  1906-8,  xi.  584. 

6  "  Syphilitic  Disease  of  the  Eye  and  Ear,"  1863,  1. 


CHOEOIDITIS  193 

of  acute  illness  develops  iritis,  it  is  almost  undeniable  evidence 
of  congenital  syphilis. 

With  regard  to  the  effect  of  treatment  Hutchinson  says  :  "I 
have  but  limited  faith  in  mercurial  treatment  as  a  prophylactic 
either  against  this  or  any  other  of  the  phenomena  of  infantile 
syphilis." 

He  states  that  in  many  of  the  cases  the  patients  had  been 
previously  under  treatment  by  mercury  for  other  symptoms  of 
hereditary  syphilis,  and  that  in  five  instances  in  the  adult  he 
had  seen  syphilitic  iritis  set  in  during  actual  ptyalism  from 
mercury. 

It  is  here  convenient  to  state  my  conviction,  that  neither 
mercury  nor  any  of  the  salvarsan  compounds  will  prevent  the 
onset  of  interstitial  keratitis,  or  the  other  eye  symptoms  of 
congenital  syphilis  ;  it  is  hardly  to  be  expected,  then,  that  they 
will  bring  about  a  cure  when  the  disease  is  established. 

I  have  seen  an  infant,  the  child  of  a  trained  nurse,  which 
developed  severe  interstitial  keratitis  at  the  early  age  of  three 
years,  and  this  in  spite  of  the  fact  that  it  had  been  given 
continuous  treatment  by  mercurial  inunctions  since  within  a 
week  of  its  birth.  ^/ 

Further,  interstial  keratitis  in  congenital  syphilis  invariably 
affects  both  eyes,  but  it  is  usual  for  there  to  be  a  greater  or 
lesser  interval  between  the  time  of  their  involvement ;  never- 
theless it  is  undoubted,  that  no  form  of  treatment,  however 
vigorously  prosecuted,  will  prevent  the  onset  of  the  disease  in 
the  second  eye. 

In  the  acquired  disease  iritis  comes  on  during  the  secondary 
stage,  usually  within  six  months  of  the  date  of  infection.  In 
many  cases  it  shows  characteristic  features  in  that  buff-coloured 
nodules  appear  along  the  pupillary  margin  of  the  iris  or,  less 
often,  at  its  base,  but  never  in  the  intervening  portion.  In  a 
good  many  cases,  however,  there  are  no  features  which  are  hi  -^u 
any  way  distinctive. 

Choroiditis. — This  occurs  in  two  chief  forms,  in  the  one  there 
are  numerous,  quite  isolated  spots  or  areas,  which  are  most 
numerous  towards  the  periphery  of  the  fundus.  During  their 
active  stage,  owing  to  coincident  exudation  into  the  vitreous, 

13 


194  SYPHILIS 

they  are  seen  dimly  as  very  hazy,  yellowish  areas  ;  later,  when 
complete  subsidence  has  occurred,  they  remain  as  discrete, 
circular,  white  spots,  surrounded  and  partly  overlain  by  pig- 
ment which  has  undergone  proliferation.  This  variety  is  seen 
most  often  in  congenital  syphilis,  and  is  often  associated  with 
interstitial  keratitis. 

The  second  variety  also  is  seen  most  often  in  congenital 
syphilis.  In  it  there  is  a  widespread,  diffuse  involvement  of 
the  choroid,  the  overlying  retina  being  seriously  involved,  and 
consequently  the  term  chorio-retinitis  becomes  appropriate. 
After  the  acute  stage  has  subsided,  the  fundus  is  seen  to  be 
the  seat  of  a  widespread,  pigmentery  disturbance,  with  irregular 
areas  of  choroidal  destruction  scattered  over  it  ;  more  or  less 
optic  atrophy  is  evident,  the  retinal  vessels  may  be  greatly 
constricted,  the  retina  invaded  by  pigment  which  has  migrated 
into  it  from  the  choroid,  and  impairment  of  sight,  which  is 
often  especially  evident  at  night,  is  usually  present. 

Retinitis. — In  a  few  cases  the  retina  is  so  much  chiefly  and 
primarily  implicated  that  the  term  syphilitic  retinitis  is  properly 
applied  to  it.  The  whole  retina,  but  especially  its  central  part, 
is  cedematous,  swollen,  and  opaque -looking  ;  the  disc  edges  are 
completely  obscured ;  the  veins  are  much  engorged  and  tortuous ; 
haemorrhages  are  scanty. 

The  onset  of  the  symptoms  is  often  rapid,  and  there  is 
profound  loss  of  sight. 

As  the  condition  subsides,  slight  pigmentary  infiltration  of 
the  retina  is  seen,  the  vessels  are  reduced  in  size  and  show 
marked  peri  vascular  white  lines,  the  disc  is  partly  atrophied, 
and  day  blindness  may  occur. 

The  condition  is  very  resistant  to  treatment. 

Gummatous  infiltration  of  the  tarsus,  sclerotic,  bones  and 
periosteum  of  the  orbit  may  occur,  and  in  the  last  case  proptosis, 
ocular  paralysis  and  lachrymal  obstruction  may  result  by 
involvement  of  the  corresponding  structures. 

Optic  Disc  Changes. — Active  changes  may  occur  in  the  optic 
disc  as  a  result  of  syphilis,  in  the  entire  absence  of  any  discover- 
able clinical  evidence  of  intra-cranial  involvement,  either  at  the 
time  or  subsequently.  Such  cases  are  probably  due  either 


Left 


Seel 


Kight 


Seel 


FIG.  66. — Sections  through  the  optic  nerve  at  the  levels  indicated  to  show  the 
distribution  of  the  degeneration  of  the  fibres  ;  in  the  small  diagrams  alongside 
the  best  preserved  bundles  are  indicated.  There  is  diffuse  degeneration  in  the 
right  nerve,  chiefly  in  the  uncrossed  fasciculi ;  in  the  left  degeneration  is  less 
diffuse  and  is  chiefly  confined  to  crossed  fasciculi,  the  condition  therefore 
probably  caused  an  original  homonymous  hemianopsia.  From  a  man  aged 
thirty-three  who  died  of  a  malignant  hypophysial  adenoma  with  an  interpedun- 
cyst.  His  sight  had  been  failing  for  thre 


cular 

blind  in  the  right  eye  for  six  months. 


ree  years  and  he  had  been  totally 
(After  Cushing  and  Walker.) 


OPTIC  DISC  CHANGES  195 

.  .  .  L^  <-< 

to  a  syphijitic^, process  in  the  nerve  itself,  or  in  some  cases  to 

gummatous  periostitis  at  the  apex  of  the  orbit.  They  are  not 
uncommonly  unilateral,  and  slight,  though  definite  pain  is 
complained  of  on  moving  the  affected  eye  ;  two  important  points 
of  distinction  from  the  changes  which  develop  in  the  optic  discs 
as  a  result  of  cerebral  disease. 

The  onset,  in  my  own  experience,  is  within  liyear  of  infection, 
and  in  two  instances  I  have  seen  it  come  on  whilst  the  patient 
was  actually  undergoing  a  course  of  antisyphilitic  treatment. 
In  one  of  them  tlTe~"second  eye  became  involved  six  weeks  after 
the  first,  whilst  the  patient  was  in  hospital  undergoing  a 
continuation  of  the  treatment  on  account  of  the  first  eye. 

As  showing  the  frequency  with  which  changes  in  the  optic 
papilla  are  associated  with  cerebral  syphilis,  the  following 
figures  are  compiled  from  Uhthoff's  100  cases  :— 

In  fifty-two  of  them  the  fundi  were  normal  ;  in  eight,  other 
changes  were  present,  and  in  forty  there  were  changes  in  the 
optic  discs.  Of  these  forty,  fourteen"exhibited  a  primary  optic 
atrophy,  and  in  the  remaining  twenty-six  either  papillcedema 
or  atrophy  secondary  to  it  was  present. 

Optic  Atrophy. — Syphilis  is  responsible  for  a  very  large 
proportion  of  all  cases  of  optic  atrophy,  whether  this  occurs 
as  a  complication  of  general  paralysis,  tabes,  meningitis,  gumma 
of  the  brain  or  local  eye  disease. 

In  addition,  however,  it  is  probable  that  primary  optic 
atrophy  may  occur  as  an  independent  parasyphilitic  manifesta- 
tion, and  quite  apart  from  other  parasyphilitic  disease. 

Cases  are  reported  by  Read  Wilson  ;  l  and  Mclntosh, 
Fildes,  Head  and  Fearnsides,2  in  their  article  on  parasyphilis 
of  the  nervous  system,  say  :  "  No  one  can,  therefore,  doubt 
that  primary  optic  atrophy  can  occur  as  a  parasyphilitic 
phenomenon  alone,  or  in  combination  with  primary  degenera- 
tion of  the  lateral  or  posterior  columns  of  the  spinal  cord." 

Paralysis  of  the  Ocular  Nerves. — The  most  common  cause  of 
paralysis  of  the  ocular  nerves  in  syphilis,  if  parasyphilis  be 
excluded,  is  probably  a  direct  involvement  of  them  by  gumma- 
tous disease  in  the  orbit,  or  a  basal  syphilitic  meningitis. 

J-  Brain,  1912,  xxxv.,  173. 
2  Brain,  1913 — 14,  xxxvi.,  1. 

13—2 


196  SYPHILIS 

Less  frequent  causes  are  cerebral  gumma,  syphilitic  neuritis 
of  the  nerve  trunks,  or  nuclear  degeneration  from  disease  of 
the  supplying  vessels. 

Whilst  ocular  palsies  arise  in  many  different  ways,  some  form 
of  syphilitic  disease  is  by  a  long  way  the  most  common  single 
cause.  Thus;  of  141  cases  of  ophthalmoplegia  collected  by 
Collins  and  Wilde,1  at  least  one-third  were  due  to  syphilis. 

Uhthoff  2  analysed  minutely  100  cases  of  cerebral  syphilis  ; 
amongst  these  the  third  nerve  was  involved  in  thirty -four  cases  : 
fifteen  times  unilaterally,  fifteen  times  bilaterally,  and  four 
times  unilaterally  with  crossed  hemiplegia  due  to  involvement 
of  the  crus  cerebri  in  the  interpeduncular  space.  The  sixth 
nerve  was  involved  sixteen  times  :  eleven  times  bilaterally, 
four  times  unilaterally,  and  once  unilaterally  with  crossed 
hemiplegia.  The  fourth  was  involved  unilaterally  four  times  and 
bilaterally  once.  The  fifth  was  involved  in  fourteen  cases,  all 
unilaterally. 

In  some  cases  the  third  nerve  is  only  partly  affected,  and  then 
ptosis  is  the  commonest  manifestation. 

Of  sixty-two  cases  of  complete  third  nerve  paralysis  in 
cerebral  syphilis  Uhthoff  found  that  it  was  :— 

Unilateral,  without  hemiplegia,  in  nineteen  cases. 
Bilateral,  without  hemiplegia,  in  thirty-five  cases. 
Bilateral  with  crossed  hemiplegia  in  eight  cases. 

Of  seventeen  of  these  cases  which  came  to  autopsy  in  nine 
years  the  optic  papilla,  nerves,  chiasma  or  tracts  were  involved 
in  fourteen.  . 

Ocular  palsies  are  rarely  seen  in  congenital  syphilis,  but  the 
nerves  may  be  involved  in  a  periostitis,  or  they  may  be  the  seat 
of  a  gummatous  infiltration.  Lawford,  in  reporting  two  cases,3 
says  :  "  Very  few  authors  to  whose  works  I  have  referred 
mention  the  occurrence  of  ocular  palsies  in  connection  with 
congenital  syphilis."  Cases  are  reported  by  Wells,  Nettleship,4 
Hutchinson.5 

1  Brit.  Med.  Journ.  1891,  ii.,  475. 

2  v.  Graefe's  Archiv.  f.  Ophth.,  1894,  xl.,  i..  43. 

3  Ophth.  Review,  1890,  ix..  97. 

*  Trans.  Path.  Soc.,  1881,  xxxii.,  13. 
5  "  Syphilis,"  1887,  208. 


GROSS  TUBERCULOUS  LESIONS 
GROSS   TUBERCULOUS   LESIONS. 


197 


Gross  tuberculous  disease  of  the  eye  is  occasionally  seen, 
due  to  a  blood  infection  of  some  part  of  the  uveal  tract. 

Severe  irido-cyclitis  occurs,  there  is  exudate  into  the  vitreous 
which  causes  a  yellowish  pupillary  reflex,  the  retina  is  detached, 
and  ultimately  the  sclerotic  is  softened  and  gives  way  before 
the  intra-ocular  contents,  and  complete  destruction  of  the  eye 
ensues  (Fig.  67). 

Besides  these  cases,  however,  some  observers  attribute  many 


FIG.  67. — Showing  gross  tuberculous  disease  of  the  iris  and  ciliary  body. 
The  anterior  chamber  is  filled  with  exudate  and  the  mass  of  granula- 
tions (A)  have  perforated  the  globe. 

of  the  numerous  cases  of  gross  solitary  choroiditis  that  are  seen 
to  the  tubercle  bacillus,  but,  as  it  seems  to  me,  on  quite 
insufficient  grounds. 

This  form  of  disease  always  gets  well  in  the  course  of  time, 
leaving  a  large  pigmented  scar  in  the  choroid.  Apart  from 
miliary  choroidal  tubercles,  I  believe  tuberculous  disease  of  the 
eye  is  quite  rare. 

Peter  l    examined    ophthalmoscopically    the    eyes    of    320 

1  Amer.  Jour,  of  Ophth.,  1916,  xxxiii.,  105. 


198  MI  LI  ART  TUBERCULOSIS 

patients  in  the  last  stages  of  pulmonary  tuberculosis,  and  in 
no  single  case  did  he  find  a  tuberculous  lesion  of  the  uveal 
tract. 

I  suggest  the  following  as  a  probable  explanation  of  some 
of  these  cases>  though  I  can  offer  no  direct  evidence  in  its 
support : — 

That  at  times  minute  emboli  occur  in  the  blood  stream  of 
individuals  in  health,  or  who  are  at  most  but  slightly  indisposed. 

Such  minute  emboli  may  lodge  in  the  vessels  of  any  of  the 
tissues  of  the  body,  except  the  eye — not  excluding  most  parts 
of  the  brain  and  heart — without  giving  rise  to  discoverable 
lesions,  or  even  disturbing  symptoms  ;  should  such  an  embolus, 
however,  lodge  in  any  of  the  intra-ocular  vessels,  whether  those 
of  the  retina  or  choroid,  the  resulting  obscuration  of  vision 
cannot  be  overlooked,  and  so  the  actual  lesion  which  is  visible 
in  this  organ  alone  of  the  whole  body  is  discovered. 

Such  an  occurrence  in  the  retinal  artery  is  well  recognised 
under  the  term  "  embolism  of  the  central  retinal  artery,"  and 
it  is  significant  that  in  many  or  most  such  cases  no  source  of  the 
embolism  is  discovered,  and  I  suggest  that  many  of  the  cases 
of  choroidal  lesions  which  by  some  are  attributed  to  the  tubercle 
bacillus,  are  in  fact  brought  about  by  the  lodgment  of  an 
embolus  of  unknown  source  in  one  of  the  vessels  of  the  choroid. 

*      MILIARY   TUBERCULOSIS. 

In  miliary  tuberculosis  tubercles  are  often  developed  in  the 
choroid  and  are  then  of  great  diagnostic  value,  seeing  however, 
that  they  are  by  no  means  always  present  in  this  disease,  their 
absence  is  of  little  negative  value. 

Incidence  in  Tuberculous  Meningitis. — There  is  an  extra- 
ordinary variation  in  the  estimates  of  various  authors  as  to  the 
frequency  of  choroidal  tubercles  in  this  condition ;  some  have 
found  them  in  all  cases,  and  some  have  said  that  they  seldom  if 
ever  occur.  These  wide  discrepancies  arise  from  two  causes. 

First,  a  thorough  examination  of  the  fundus  in  the  subjects 
of  this  disease  is  often  difficult  and  unsatisfactory,  so  that 
figures  based  on  ophthalmoscopic  investigation  alone  are  sure 
to  indicate  an  incidence  which  is  less  than  the  truth. 


CHOROIDAL  TUBERCLES  199 

Secondly,  choroidal  tubercles  are  developed  late  in  the 
disease ;  consequently,  unless  the  stage  at  which  the  examinations 
are  made  is  taken  into  account,  the  estimates  are  sure  to  be 
misleading  ;  the  largest  number  of  ^ases  would  be  found  were 
all  the  examinations  made  immedia/Ee1y~betore  death. 

Tooke  found  seven  cases  of  choroidal  tubercles  amongst 
seventy-three  cases  of  tuberculous  meningitis,  but  in  no  case  were 
they  present,  he  states,  earlier  than  three  days  before  death. 

Choroidal  tubercles  are  readily  seen  by  the  aid  of  a  lens  or 
indeed  with  the  naked  eye  in  the  post-mortem  room  ;  with  a 
view  therefore  of  obtaining  an  absolute  estimate  of  their  fre- 
quency, I  have  examined  the  eyes  of  thirty-three  patients 
suffering  from  miliary  tuberculosis  in  this  way  ;  all  but  two  of 
them  had  tuberculous  meningitis.  The  average  age  of  the 
patients  was  six  years,  the  oldest  being  twenty  and  the 
'  youngest  five  months.  Of  these  thirty -three  patients,  ten,  i.e., 
29  per  cent.,  had  one  or  more  tubercles  in  one  or  both  eyes. 
It  may,  then,  be  said  that  they  occur  in  rather  less  than  one- 
third  of  cases.  - 

I  have  seen  a,  single  tubercle,,  present  in  one  eye  only,  with 
none  in  the  opposite  eye,  and  I(have  counted  as  many  as  forty- 
seven  discrete  tubercles  in  a  single  eye) 

In  the  great  majority  of  cases  in  which  tubercles  are  present 
in  the  choroid,  they  will  also  be  found  in  the  meninges,  but  this 
is  not  necessarily  so.  I  have  seen  two  cases  where  choroidal 
tubercles  were  present,  in  which  the  viscera  in  general  were 
plentifully  scattered,  but  none  were  discoverable  on  most 
careful  examination  of  the  meninges. 

Whilst  in  general  it  is  true  that  when  choroidal  tubercles 
develop  meningitis  is  also  present,  and  so  it  comes  about  that 
their  presence  usually  precedes  death  by  a  short  time  only, 
nevertheless  in  those  rarer  instances  of  miliary  tuberculosis 
where  choroidal  tubercles  develop  but  the  meninges  are  free  of 
disease  either  throughout  or  until  a  later  stage,  the  presence  of 
tubercles  in  the  choroid  may  precede  death  by  a  longer  period. 

The  longest  time  that  I  can  be  sure  of  having  seen  them  before 
death  is  thirty4wo  days,  but  no  doubt  this  period  may  be 
exceeded. 


200 


CHOROIDAL  TUBERCLES 


FIG.  68.- — A  section  through  a  large  choroidal  tubercle  from  a  case  of 
tuberculous  meningitis.  In  the  centre  is  an  hsemorrhagic  area,  and 
several  giant  cells  (B)  are  seen.  During  life  the  tubercle  had  some- 
what the  appearance  depicted  in  Fig.  69,  i.e.,  it  was  pale  in  the 
centre  with  a  pigment  fringe  around  ;  it  gave  the  impression  that 
the  tubercle  was  so  prominent  that  it  had  protruded  through  the  pig- 
ment epithelial  layer  of  the  retina  so  that  the  ragged  free  edge  of  the 
latter  appeared  as  a  fringe.  The  edge  of  this  pigment  lay  is  seen  (^4). 


FIG.  69. — A  drawing  made  during  life  of  the  choroidal  tubercle  shown 
in  section  above.  A.  Points  to  the  pigmentary  fringe  caused  by 
the  protrusion  of  the  summit  of  the  tubercle  through  the  pigment 
epithelium. 

Barlow  *•  states  that  in  sixteen  cases  of  tubercle  of  the  choroid 
three  showed  the  presence  of  tubercles  in  other  organs,  but  none 
in  the  meninges. 

1  Allbutt's  "System  of  Medicine,"  1910,  vii.,  217. 


CEREBRO-SPINAL  MENINGITIS  201 

Other  cases  showing  this  to  be  true  are  reported  by  Randolph 
and  Schmeisser,1  Lawford  2  and  Warner.3 

It  is  evident  that  individual  tubercles  are  rapidly  formed.  I 
have  seen  one  become  visible  to  the  ophthalmoscope  in  five 
hours  ;  Warner  has  seen  them  develop  within  a  day,  and 
Marples  4  has  seen  one  develop  within  four  hours.  Of  six  cases 
examined  histologically  by  Lawford,  two  showed  the  presence 
of  plentiful  bacilli,  but  he  was  unable  to  find  them  in  the 
remaining  four  cases.  Their  average  diameter  is  about  1  mm., 
but  they  vary  a  good  deal  in  size. 

Some  slight  oedema  of  the  optic  disc,  with  blurring  of  its  edges, 
is  often  seen  in  tuberculous  meningitis,  but  a  swelling  of  the 
nerve  head  at  all  comparable  with  the  papillcedema  of  a  cerebral 
tumour  is  rarely  seen.  Tooke  states:  "Early  inflammatory 
changes  about  the  nerve,  described  by  some  as  perineuritis, 
by  others  as  a  perineural  oedema,  were  recorded  in  thirty  out 
of  the  seventy-three  cases  examined."  Hughlings  Jackson  5 
says  :  "In  tuberculous  meningitis  the  swelling  is  slight,  not 
extensive,  it  is  even  and  merges  into  the  fundus  ;  the  disc  is 
succulent  -  looking  ;  there  is  not  time  possibly  for  further 
development.' 

CEREBRO-SPINAL   MENINGITIS. 

The  ocular  lesions  associated  with  this  disease  may  be 
divided  into  those  which  are  to  be  seen  within  the  eye  itself, 
and  lesions  which  manifest  themselves  as  functional  defects. 

Different  epidemics  show  a  varying  incidence  of  ocular 
lesions. 

The  most  important  changes  which  occur  are  :— 

(1)  Metastatic    intra-ocular    infections,    i.e.,    conjunctivitis. 

iritis,  choroiditis,  retinitis  or  panophthalmitis. 

(2)  Papilloedema. 

(3)  Amaurosis  without  ophthalmoscopic  signs. 

(4)  Ocular  palsies  and  nystagmus. 

1  Trans.  Am.  Oph.  Soc.,  1916,  xiv.,  589. 

2  Trans.  Oph.  Soc.  U.K.,  1886,  vi.,  351. 

3  Trans.  Oph.  Soc.  U.K.,  1883,  iii.,  127. 

4  "Ophthalmoscope,"  1912,  x.,  561 

5  Trans.  Oph.  Soc.  U.K.,  1881,  i.,  64. 


202  CEREBRO-SPINAL  MENINGITIS 

Incidence. — Amongst  100  cases  Heine1  found  ocular  com 
plications  in  twenty-seven ;  Vinsonneau  2  found  two  cases 
amongst  twenty  of  the  disease  ;  Randolph  3  found  seven  cases 
of  well-marked  intra-ocular  change  amongst  thirty-five  cases, 
and  in  nineteen  other  cases  there  was  marked  engorgement  of 
the  retinal  veins,  and  he  states  that  this  last  was  a  feature  of 
the  particular  epidemic. 

(1)  Met  astatic  Infections. — Of  the  metastatic  infections  the 
least  important  is  a  conjunctivitis  which  usually  is  of  no  great 
severity,  although  it  has  occasionally  been  serious  ;   in  two  out 
of  six  cases  McKee  4  isolated  the  meningococcus  from  the  dis- 
charge. 

The  -intra-ocular  metastases  are  much  more  serious  ;  they  are 
not  common  nor  are  they  excessively  rare ;  they  occur  in 
sporadic  cases  as  well  as  in  epidemics.  At  first  there  is  a 
yellowish  reflex  from  the  pupil  owing  to  infiltration  of  the 
vitreous,  iridic  adhesions  quickly  form,  and  gradual  shrinkage 
of  the  globe  occurs.  To  the  condition  of  the  eye  which  results, 
the  term  "  pseudo-glioma  "  is  sometimes  applied  from  its 
simulating  the  appearance  of  a  glioma  of  the  retina.  The 
condition  is  usually  unilateral,  and  according  to  Randolph  is 
most  common  in  the  right  eye.  The  signs  of  inflammation 
of  the  eye  are  slight,  vision  is  always  lost. 

(2)  Papilloedema  is  not  of  frequent  occurrence  :    Randolph 
found  it  in  17  per  cent,  of  his  cases  ;   Batten  5  says  optic  neuritis 
is  rare  ;    Barlow  and  Lees  6  found  three  cases  only  amongst 
forty -two  cases  of  the  disease. 

Terrien  and  Boudier  7  examined  three  cases  where  death 
occurred  within  the  first  week,  and  in  whom  the  only  ophthal- 
moscopic  sign  was  hypersemia  of  the  papilla. 

Their  conclusions  were  : — 

(1)  Meningeal  infection  spreads  along  the  sheath  of  the  optic 

1  Berlin,  Klin.  Wochenachr.,  1905,  xlii.,  772. 

2  Archiv.  cTOphth.,  1914,  xxxiv  ,  349. 

3  Bullet,  of  the  Johns  Hopkim  Hosp.,  1893,  iv.,  No.  32,  59. 

4  Ophth.  Record,  1908,  xvii  ,  438. 

5  Garrod  and  Thursfield,  "Diseases  of  Children,"  1913,  747. 
«  Quoted  Batten;  Albutt's  "Syst.  of  Med.,"  1910,  vii.,  174. 
:  Archiv.  d'Ophth.,  1909,  xxix.,  301,  and  1910,  xxx.,  214. 


TEMPORARY  AMAUROSIS  203 

nerve  in  the  same  way  and  at  the  same  period  as  on  the 
surface  of  the  brain  and  spinal  cord. 

(2)  It  begins  as  an  inflammation  of  the  arachnoid  and  pia, 

and  may  go  on  to  resolution  or  provoke  lesions  in  the 
parenchyma  of  the  optic  tracts. 

(3)  The  process  is  a  diffuse  perineuritis  extending  the  whole 

length  of  the  optic  tracts,  but  is  most  intense  at  the 
optic  canal.  In  some  cases  the  nerve  suffers  from 
a  secondary  interstitial  neuritis  leading  to  disappearance 
of  nerve  bundles  and  optic  atrophy.  This  secondarily 
developed  interstitial  neuritis  may  explain  cases  in 
which  blindness  has  supervened  during  convalescence 
without  ophthalmoscopic  change. 

(3)  Amaurosis   without  Ophthalmoscopic  Signs. — Dr.   Hugh 
Thursfield  tells  me,  from  his  large  experience  at  the  Great 
Ormond  Street  Children's  Hospital,  that  a  very  large  proportion 
of  infants  show  evidence  of  great  loss  of  sight  during  the  illness. 
The  pupils  remain  active  to  light  stimulus  and  the  fundi  are 
normal. 

There  is  no  doubt  that  many  of  the  cases  of  "  acute  amaurosis 
of  infants  "  (p.  249)  are  of  this  nature. 

In  the  majority  of  cases  sight  is  completely  restored,  some- 
times quite  suddenly,  and  this  may  happen  after  the  blindness 
has  persisted  for  many  weeks. 

Batten  *  says  :  "  The  blindness  which  is  present  in  this 
disease  is  a  striking  feature ;  it  is  often  present  as  an  early 
manifestation  and  is  complete.  It  is  not  due  to  optic  neuritis 
or  atrophy  ;  optic  neuritis  is  but  rarely  seen,  the  optic  discs  and 
the  vessels  of  the  fundus  are  generally  perfectly  normal. 

"  The  child  will  sometimes  remain  perfectly  blind  after 
general  recovery  has  taken  place ;  in  some  cases  the  blindness 
will  persist  for  many  weeks  and  then  sight  may  be  recovered 
completely  ;  the  longest  period  that  I  have  seen  a  child  blind 
and  recover  good  vision  is  four  months." 

(4)  Ocular  Palsies  and  Nystagmus. — Paralysis  of  some  of  the 
ocular  muscles  is  of  common  occurrence,  and  according  to 

1  Garrod  and  Thursfield,  "  Diseases  of  Children,"  1913,  749. 


204  PYMMIA   AND  SEPTICAEMIA 

Randolph  l  it  most  frequently  affects  the  right  eye ;  thus,  of 
thirty-five  cases,  divergent  strabismus  was  present  in  eight, 
and  in  each  case  it  was  the  right  eye  that  was  affected  ;  he 
points  out  that  this  tendency  to  right-sided  lesions  has  been 
noted  by  others. 

The  paralyses  are  probably  due  to  the  involvement  of  the 
nerve  trunks  at  the  base  of  the  brain. 

Nystagmus  is  sometimes  seen.  In  some  severe  cases  corneal 
ulceration  develops  owing  to  incomplete  closure  of  the  lids,  the 
lower  part  of  the  cornea  remaining  exposed,  and  blinking  being 
largely  in  abeyance  In  such  cases  it  is  the  exposed  part  only 
which  becomes  ulcerated,  and  it  consequently  has  a  horizontal 
upper  edge,  corresponding  with  the  lower  margin  of  the  upper 
lid,  both  eyes  are  usually  affected  ;  perforation  may  occur.  If 
the  child  survives,  the  resulting  bilateral  nebulae  with  sharp-cut 
horizontal  upper  limits,  are  left  to  tell  the  tale  to  those  who  can 
read,  of  a  serious  and  prolonged  illness,  during  which  the  patient 
lay  in  an  unconscious  state  with  the  eyes  only  partly  closed. 

PYAEMIA   AND   SEPTICAEMIA. 

In  these  conditions  where  there  is  a  generalised  infection  of 
the  b^pod  whatever  the  original  source,  retinal  hemorrhages  are 
almost  invariably  present  towards  the  end  of  the  illness,  and, 
as  in  infective  endocarditis,  they  are  probably  caused  by  the 
lodgment  of  infected  emboli  in  the  retinal  capillaries. 

These  emboli  may,  as  in  the  latter  disease,  lead  to  uni-  or 
bi-lateral  panophthalmitis,  with  consequent  complete  destruc- 
tion of  the  eyeball,  sometimes  without  the  endocardium  being 
involved. 

There  is  little  evidence  of  acute  inflammation  of  the  eye,  the 
iris  becomes  discoloured,  there  is  fibrinous  exudation  into  the 
pupil,  a  yellowish  reflex  is  obtained  from  the  infected  vitreous 
giving  rise  to  an  appearance  to  which  the  term  pseudo-glioma  is 
applied,  and  the  eye  shrinks  and  becomes  blind  ;  as  mentioned 
elsewhere,  this  complication  may  occur  in  post-basic  meningitis, 
it  is  also  seen  in  other  forms  of  meningitis. 

1  Bullet,  of  the  Johns  Hopkins  Hosp.,  1893,  iv.,  No  32,  59. 


INFECTIVE  ENDOCARDITIS  205 

Flemming  x  collects  forty -two  of  this  type  of  case,  of  whom 
only  six  died,  and  comments  upon  the  comparative  rarity  of  a 
fatal  termination. 

Bull  2  reports  six  such  cases  occurring  during  pneumonia. 

INFECTIVE   ENDOCARDITIS. 

The  common  lesion  of  the  eye  in  this  disease  is  the  presence 
of  haemorrhages  into  the  retina,  and  these  are  so  common  as  to 


FIG.  70. — Showing  the  lodgment  of  an  embolus  in  a  branch  of  the  retinal 
artery  in  infective  endocarditis.  From  a  man  aged  twenty-seven, 
under  the  care  of  Dr.  Langdon  Brown,  who  had  been  under  obser- 
vation in  hospital  for  five  weeks.  He  awoke  an  hour  after  having 
gone  to  sleep  to  find  he  could  not  see  with  his  right  eye.  A.  indicates 
the  position  of  the  embolus,  and  the  arrows  show  the  course  of  the 
"visible  streaming"  of  the  blood  in  the  arteries,  the  direction  of 
flow  being  reversed  in  one  of  them. 

be  usual.  They  occur  at  the  same  time  as  the  petechial 
haemorrhages  which  are  so  frequent  in  other  parts,  and  have 
little  value  in  diagnosis  or  prognosis.  Of  twenty-four  cases  of 
my  own,  sixteen  were  males  and  eight  were  females,  the 
average  age  being  27-7  years.  In  eight  of  these  cases  no 
haemorrhages  were  discoverable  at  the  time  of  examination,  but 
in  very  few  cases  does  the  retina  remain  free  of  haemorrhages 
right  up  to  the  time  of  death. 

1  Trans.  Ophth.  Soc..  U.K.,  1900,  xx.,   135. 

2  Trans.  Amer.  Ophth.  Soc..  1900,  ix.,  316. 


206  INFECTIVE  ENDOCARDITIS 

The  haemorrhages  are  usually  small  and  scattered  and  are 
often  roughly  circular  in  outline,  they  do  not  show  the  same 
preference  for  the  central  regions  as  do  most  other  forms  of 
haemorrhage,  no  doubt  from  the  fact  that  they  represent 
minute  emboli  whose  point  of  lodgment  will  depend  upon  their 
size. 

Sometimes  they  appear  as  ordinary  flame-shaped  haemor- 
rhages in  the  region  of  the  disc,  sometimes  they  are  large  and 
infiltrate  the  peri  vascular  sheaths,  sometimes  they  overlie  the, 
macula  and,  like  other  subhyaloid  haemorrhages,  cause  ery- 
thropsia,  and  sometimes  they  have  pale  nucleus-like  centres  such 
as  are  seen  most  frequently  in  leukaemia. 

Occasionally  other  ocular  complications  arise.  Sometimes 
emboli  appear  to  lodge  in  the  ciliary  arteries  greatly  disturbing 
the  choroidal  circulation  ;  there  is  rapid  and  great  loss  of 
sight,  the  retina  has  a  raised  and  uneven  surface,  is  very  hazy 
and  cedematous,  and  bleedings  occur  in  its  substance ;  if  the 
patient  survives,  extensive  pigmentary  changes  develop  in 
the  choroid. 

Sometimes  there  is  a  sudden  serious  loss  of  sight  from  lodg- 
ment of  an  embolus  in  the  central  retinal  artery  or  one  of  its 
large  branches. 

Fig.  70  was  drawn  from  a  man  of  twenty -seven  who  developed 
an  embolism  in  a  branch  of  his  right  retinal  artery  whilst  he  was 
in  hospital  for  infective  endocarditis.  At  ten  o'clock  he  was 
as  well  as  usual;  he  awoke  at  eleven  o'clock  with  "  a  funny 
feeling  "  in  his  head  and  found  he  couldn't  see  with  his  right 
eye.  Fifteen  hours  later  there  was  the  milky  cedematous 
appearance  of  coagulation  necrosis,  affecting  the  part  of  the 
retina  supplied  by  the  artery.  The  arteries  were  greatly 
reduced  in  size,  and  the  contained  column  of  blood  was 
broken  up  into  short  blocks  of  red  cells  with  intervening 
short  intervals  of  clear  plasma,  the  so-called  "  cattle  truck 
appearance."  The  blood  could  thus  be  seen  to  be  travelling 
along  the  arteries  in  a  slow  stream  in  the  direction  indicated  by 
the  arrows ;  in  one  branch  it  was  travelling  in  a  reversed  direction. 

It  seemed  that  a  collateral  circulation  between  the  artery 
and  the  next  below  was  present,  and  the  blood  therefore  came 


FACIAL  ERYSIPELAS  207 

down  this  vessel  and  was  distributed  along  the  other  branches 
as  indicated.  This  slow  circulation  was  easily  arrested  by 
slight  pressure  upon  the  eyeball. 

In  most  cases  the  emboli  show  no  signs  of  being  infective, 
but  it  happens  occasionally  that  panophthalmitis  with  destruc- 
tion of  the  eye,  follows  upon  the  lodgment  of  an  embolus  in 
which  active  organisms  are  present. 

FACIAL  ERYSIPELAS   AS   A  CAUSE   OF   BLINDNESS. 

In  some  cases  of  facial  erysipelas  the  infection  spreads  to  the 
orbital  tissues,  producing  orbital  cellulitis  and  complete  loss  of 
vision  in  one  or  both  eyes,  and  a  similar  result  may  ensue  from 
cellulitis  of  the  orbit  produced  in  other  ways.  It  often  happens 
that  the  actual  onset  of  the  blindness  is  overlooked  from  the 
fact,  that  during  the  acute  stage  the  lids  are  so  greatly  swollen 
that  it  is  not  possible  to  expose  the  cornea,  and  it  is  only 
when  this  swelling  has  subsided  that  the  loss  of  sight  is  revealed. 
There  is  marked  proptosis,  chemosis  and  limitation  of  movement. 

Knapp  l  examined  the  fundi  of  a  case  throughout ;  he  found 
that  the  vision  was  completely  lost  in  two  days,  and  that 
haemorrhages  were  freely  scattered  all  over  the  fundi  as  a  result 
of  thrombosis  in  the  retinal  artery  and  veins.  He  showed  2  that 
of  thirty -five  cases  the  mortality  was  29  per  cent.,  and  of 
those  surviving,  totaP  blindness  in  both  eyes  occurred  in  16 
per  cent.,  and  in  one  eye  in  60  per  cent.  Other  cases  are  reported 
by  Leber,3  Williams,4  Coggin,  and  others. 

GONOCOCCAL   IRITIS. 

Having  regard  to  the  prevalence  of  gonorrhoea,  iritis  must 
be  considered  an  uncommon  complication  of  it ;    at  the  same 
time  it  is  probably  the  commonest  cause  of  a  primary  acute  "^ 
iritis  in  an  adult  male. 

It  occurs  only  in  the  presence  of  a  bloocT  infection,  and, 
consequently,  those  who  are  the  subject  of  a  first  attack  are 

1  Trans.  Amer.  Opkth.  Soc.,  1883,  iii.,  573. 

2  ArcUv.  ofOphth.,  1884,  xiii.,  83. 

3  v.  Graefe's  Arehiv.f.  Ophth.,  1880,  xxvi.,  iii.,  224. 
1  Boston  Med.  and  Surg.  Jour.,    1883,  cviii.,  51. 


208  GONOCOCCAL  IRITIS 

usually  in  poor  health,  and  will  be  found  to  have  a  raised 
temperature  ;  in  recurrent  attacks,  however,  there  is  little 
constitutional  disturbance. 

On  the  other  hand,  I  have  notes  of  nine  cases  in  which  the 
gonococcus  was  obtained  in  pure  culture  in  fluid  withdrawn 
from  the  knee  joint,  in  five  of  which  the  temperature  was 
sub-normal  throughout,  and  in  a  sixth  case  it  exceeded  the 
normal  on  four  days  only. 

As  gonococcal  blood  infections  are  greatly  more  frequent 
in  males  than  in  females,  so  is  gonorrhceal  iritis  quite  unusual 
in  women. 

During  the  course  of  twelve  years  I  was  able  to  collect  131 
cases  of  primary  gonococcal  blood  infection  at  St.  Bartholomew's 
Hospital ; x  of  these  118  were  in  men,  and  thirteen  in  women. 
/It  is  unusual  to  find  a  case  of  iritis  in  which  there  has  not 
been  an  antecedent  arthritis,  and  it  was  this  association  which 
was  responsible  for  many  years  for  the  diagnosis  of  rheumatic 
iritis  which  was  usually  attached  to  these  cases. 

Iritis  does  not  occur  as  a  complication  of  rheumatic  fever. 

Date  of  Development. — In  seventy -nine  of  my  cases  in  which 
the  interval  between  the  gonorrhceal  infection  and  the  onset 
of  the  arthritis  was  noted,  it  was  less  than  a  month  in  sixty- 
eight  cases,  and  in  ten  cases  in  which  the  interval  between  the 
onset  of  the  arthritis  and  that  of  the  iritis  was  noted,  the  average 
length  of  time  was  six  weeks.  It  sometimes  happens  that  a 
patient  has  attack  after  attack  of  arthritis  without  any  evident 
iritis,  and  later,  perhaps  several  years  after  the  first  involve- 
ment of  the  joints,  iritis  is  developed  for  the  first  time. 

Recurrences  of  both  arthritis  and  iritis  are  common,  and 
apart  from  a  fresh  infection,  they  probably  indicate  the  presence 
of  organisms  lurking  in  the  genito -urinary  tract. 

Joints  Involved. — Amongst  the  131  cases  referred  to,  87  per 
cent,  had  the  knee  joint  involved,  56  per  cent,  the  ankle, 
40  per  cent,  the  shoulder,  whilst  the  joints  least  often  affected 
were  the  jaw,  sterno-clavicular,  and  sacro-iliac. 

In  the  primary  attack  of  gonorrhceal  iritis  there  is  often  a 
rather  bright  yellow,  gelatinous-looking  exudate  in  the  anterior 

1  Si.  Bartholomew's  Hospital  Reports,  1912,   xlviii.,  87. 


DYSENTERY  209 

chamber,  and  when  it  occurs,  it  is  almost  pathognomonic  of  this 
form  of  iritis  ;  it  is  not  seen  in  the  recurrences. 

In  rare  instances  the  manifestations  of  blood  infection  result 
from  gonorrhoeal  conjunctivitis,  as  in  ophthalmia  neonatorum. 

Endogenous  Conjunctivitis. — In  some  cases  of  blood  infection 
there  is  an  injection  of  the  conjunctival  and  episcleral  vessels, 
a  condition  to  which  the  term  "  metastatic  gonorrhoeal  con- 
junctivitis "  may  be  applied.  Herrfordt  l  states  that  of  2,310 
cases  of  gonorrhoea  treated  at  the  Copenhagen  Municipal 
Hospital  in  the  course  of  five  years,  this  endogenous  form 
of  conjunctivitis  occurred  in  twenty-three.  Byers  2  states 
that  in  30  per  cent,  of  such  cases  the  deeper  coats  of  the  eye 
were  involved,  and  it  is  probable  that  a  considerable  proportion 
of  cases  diagnosed  as  endogenous  conjunctivitis  have  been  cases 
of  iritis  of  so  mild  a  degree  that  the  more  conspicuous  signs, 
such  as  posterior  synechise,  have  not  developed. 

EYE   AFFECTIONS   IN   DYSENTERY. 

In  cases  of  dysentery  affections  of  the  eyes  are  by  no  means 
rare  ;  they  take  the  form  either  of  conjunctivitis  or  of  irido- 
cyclitis. 

The  conditions  are  due  to  infection  through  the  blood  stream, 
and  are  not  due  to  the  organism  having  been  conveyed  directly 
by  the  finger  to  the  conjunctival  sac.  They  may  commence  as 
a  conjunctivitis,  and  iritis  may  afterwards  be  developed.3 
In  most  cases  the  eye  symptoms  are  associated  with  arthritis 
of  some  of  the  joints,  though  this  is  not  always  so. 

Thus,  of  twelve  cases  reported  by  Cosse  and  Delord,4  eight 
suffered  from  joint  affections  ;  and  similarly  four  out  of  six 
cases  reported  by  Kiep  and  Maxwell.5  Of  thirty  cases  of 
arthritis  reported  by  Graham  6  thirteen  suffered  from  affection 
of  the  eyes. 

The  eye  symptoms  came  on  at  varying  times  after  the  onset 

1  v.  Graefe's  Archiv.f.  Ophthcd.,  1909,  Ixxii.,  344. 

2  "  Studies  from  the  Roy.  Victoria  Hospital,  Montreal,"  1908,  ii.,  No.  2,  23. 

3  Morax,  Annales  a'Oculistique,  1917,  cliv.,  45. 

4  Annales  d1  Oculistique,  1917,  cliv.,  33. 

5  British  Jour,  of  Ophth.,  1918,  ii.,    71. 

6  Proc.  Roy.  Soc.  of  Med.,  1919,  xiii.,  Section  of  Medicine,  23. 

M.O.  14 


210  TETANUS 

of  the  disease,  but  three  weeks  may  be  considered  as  about  the 
average  period  ;  the  interval  may  be  as  short  as  twelve  days 
(Kiep  and  Maxwell)  or  as  long  as  eighty-eight. days  (Graham). 

They  and  the  arthritis  are  usually  developed  at  about  the 
same  time,  sometimes  one  and  sometimes  the  other  appearing 
first  by  a  day  or  two.  There  does  not  appear  to  be  any  relation 
between  the  incidence  of  the  ocular  complications  and  the 
severity  of  the  arthritis,  for  of  Graham's  thirteen  cases  with 
ocular  complications,  in  seven  the  arthritis  is  described  as  being 
severe,  and  in  six  as  being  mild. 

All  accounts  agree  in  describing  the  conjunctivitis  as  being 
of  a  mild  type,  with  but  little  formation  of  pus  or  muco-pus.  It 
affects  the  lower  palpebral  conjunctiva  more  especially  (Cosse 
and  Delord)  ;  it  is  quickly  and  completely  cured  by  means  of 
simple  lotions.  The  iritis,  too,  is  in  general,  of  a  mild  type, 
and  if  treated  early  is  quickly  recovered  from,  but  if  neglected 
extensive  synechise  form,  and  much  trouble  may  ensue. 

The  marked  similarity  between  the  foregoing  articulo -ocular 
syndrome  and  that  which  results  from  blood  infection  in 
gonorrhoea  can  scarcely  escape  notice. 

TETANUS. 

In  a  few  cases  of  cephalic  tetanus  the  ocular  muscles  are  the 
.first  to  be  involved,  and  may  remain  the  only  manifestation  of 
tetanus  for  some  days.  These  cases  have  been  especially 
defined  by  Fromaget,1  Worms,2  and  Courtois-Suffit  and  Giroux.3 

In  many  of  the  cases  the  wound  of  infection  has  been  in  the 
orbito-ciliary  region,  an  area  which,  from  its  prominence,  is 
especially  liable  to  contused  wounds.  It  does  not  necessarily 
follow,  however,  that  should  tetanus  arise  from  a  wound  in 
this  situation,  that  it  will  be  of  the  ophthalmoplegic  type. 
,  Courtois-Suffit  says  :  "In  several  cases  of  those  in  which 
tetanic  ophthalmoplegia  was  most  clearly  defined  the  trauma 
was  in  the  eyeball." 


1  Archiv.  cTOphthal.,  1894,  xiv.,  657. 

2  Du  tetanos  bulbo-paralytiqve.     These  de  Lyon,  1906,  No.  15. 
>l  Les  formes  anormales  du  tetanos/'  Paris.  191G. 


DIPHTHERIA  211 

Sattler  l  reports  the  case  of  a  man  who  sustained  a  penetrating 
wound  of  the  cornea  by  a  dirty  wooden  arrow.  The  eye  was 
removed  within  twenty-four  hours,  but  the  patient  died  of 
acute  tetanus  in  eight  days. 

Some  only  of  the  muscles  supplied  by  the  oculo-motor  nerve 
may  be  affected,  and  in  this  case  the  levator  palpebrse  is  the 
most  frequent  ;  the  ophthalmoplegia  is  almost  always  seen 
on  the  same^side  as  the  wound,  but  the  other  side  is  involved 
later. 

The  fourth  and  sixth  nerves  are  rarely  involved,  except  in 
combination  with  the  other  nerves  of  the  orbit. 

With  regard  to  the  internal  muscles  of  the  eye,  Albert  2 
says  that  there  is  extreme  myosis,  and  that  the  pupil  does  not 
react  to  light,  but  that — at  any  rate  at  first — the  ciliary  muscle 
is  not  affected. 

DIPHTHERIA. 

f 

Diphtheritic  conjunctivitis  may  be  brought  about  by  direct 

infection  with  the  bacillus  or  as  a  result  of  extension  from  the 
nasal^cavity,  and  it  may  take  on  one  of  two  clinical  courses. 

In  the  one  variety  a  false  membrane  is  formed  on  the  con- 
junctiva, which  is  easily  peeled  off  leaving  a  raw  surface,  and 
which,  with  serum  and  simple  local  treatment,  rapidly  disap- 
pears, leaving  no  serious  residue. 

In  a  few  cases,  however,  the  tissues  of  the  lids  are  involved 
and  become  greatly  swollen  and  brawny,  a  thick  false  membrane 
forms  which  is  rapidly  reformed  after  removal,  the  cornea 
becomes  ulcerated,  and  the  eyeball  is  destroyed.  In  these  cases, 
after  removal  of  the  eye,  the  thickening  of  the  lids  andthp 
thick  washleatHer-like  false  membrane  may  persist  for  years 
in  spite  of  all  forms  of  treatment,  including  serum,  and  is  only 
cured  by  actually  dissecting  away  the  whole  of  the  diseased 
tissues,  the  conjunctival  cavity  being  thus  obliterated. 

In  these  cases  the  diphtheria  bacillus  is  not  demonstrable, 
at  any  rate  after  the  first  few  days,  and  although  the  condition 
is  here  referred  to,  some  of  these  cases  are  caused  by  the  strepto- 
coccus and  it  may  be  that  all  are  due  to  this  organism  ; 

1  Archives  of  Ophthal.,  1918,  xlvii.,  64. 

z  Etudes  sur  le  tetanos  cephalique.     Thbse  de  Lyon,  1890. 

14—2 


212  DIPHTHERITIC  PARALYSES 

it  has  to  be  remembered  that  the  xerosis  bacillus  is  a  normal 
inhabitant  of  the  conjimctival  sac,  and  that  unless  some  care 
is  exercised,  it  may  be  mistaken  for  the  Klebs-Loeffler  bacillus. 

Paralyses.—  Paralysis  of  some  of  the  ocular  muscles  is  a 
common  sequel  of  diphtheria,  it  may  affect  the  ciliary  muscle 
or  the  extrinsic  muscles. 

Next  tcTthe  sdft  palate  the  ciliary  muscle  is  probably  the 
commonest  muscle  to  be  involved  in  post-diphtheritic  paresis 
or  paralysis,  though  in  small  children  its  presence  is  not  easily 
ascertained.  Botli~eyes  are  affected.  The  date  of  onset  is 
usually  three  or  four  weeks  after  the  disease  has  commenced. 

With  regard  to  its  duration,  Ker  says  :  (*I  have  seldom  seen 
it  persist  longer  than  five  weeks,  and  its  duration  is  usually 
much  shorter  ;  perhaps  three  weeks  would  be  a  good  average^/' 

The  pupil  is  seldom  involved,  though  a  number  of  observers 

have  described  it  as  being  somewhat  dilated. 

f 

Of  the  extrinsic  muscles  the  external  recti  are  the  most 
vulnerable,  though  the  muscles  supplied  by  the  third  "nerve  are 
often  affected  ;  strabismus  and  diplopia  are  a  consequence. 

The  greatest  number  of  cases  is  seen  during  the  sixth  and 
seventh  week  after  the  onset  of  the  disease,  and  recovery  is 
usually  complete  within  twenty-one  days. 

If  an  adult  patient  is  afflicted  with  post-diphtheritic  paralysis, 
the  presence  of  a  squint  combined  with  paralysis  of  accommo- 
,. 
/^jnotor  ataxy. 

LEPROSY. 

Eye  complications  in  leprosy  are  both  frequent  and  severe. 
Of  277  lepers  examined  by  Borthen  x  12  per  cent,  were  blind  in 
one  eye  and  17  per  cent,  in  both  eyes.  Kubert  found  the  eye 
and  its  appendages  involved  in  75  per  ce'nt."  diseases,  and 
Jeanselme  and  Morax  2  found  lesions  of  the  globe  in  eleven  out 
of  fifteen  cases. 

Blindness  is  usually  due  to  involvement  of  the  uveal  tract, 
and  is  twice  as  common  in  tFe  nodular  as  in  the  anaesthetic 
variety  of  the  disease  (Borthen). 

1  "Die  Blindenverhaltnisse  bei  derlepra,"  Christiania,  1902. 

2  Annales  d1  Oculistique,  1898,  cxx.,  321. 


'/  ,.  dation  and  absent  knee  jerks  may  at  first  sight  suggest  loco- 
/^jnotor  atax. 


LEPROSY  213 

Calderaro,1  who  has  investigated  the  histology  of  the  disease, 
finds  that  bacilli  are  numerous  and  constant  along  the  margins 
of  the  lids,  but  are  rare  on  the  posterior  surface  of  the  lids  and 
are  absent  from  the  aqueous. 

He  believes  the  infection  is  conveyed  to  the  eye  by  means  of 
the  fingers  or  handkerchief,  that  the  entrance  is  through  the 
epithelium  of  the  cornea  or  conjunctiva,  and  the  episcleral 
tissues  at  the  limbus  are  the  first  to  become  involved.  Thus  he 
finds  the  first  tissue  changes  in  the  episcleral  vascular  network 
in  the  form  of  a  leprous  cell  infiltration  about  the  vessels, 
with  the  formation  of  leprous  nodules  in  the  sclerotic  ;  later 
this  infiltration  spreads  to  the  anterior  part  of  the  uveal  tract, 
iris,  ciliary  body  and  choroid. 

Jeanselme  and  Morax  state  that  the  seat  of  election  of  the 
pathological  changes  is  the  sclerotic  in  front  of  the  jnsertions 
of  the  muscles,  and  that  conjunctival  lesions  are  always  secon- 
dary to  subjacent  disease  of  the  sclerotic. 

Clinical  Manifestations.— Lagophthalmos  is  most  common  in 
anaesthetic  leprosy,  and  ulcerations  of  the  cornea  occur  which 
are  due  in  part  to  the  exposure  consequent  upon  the  lagoph- 
thalmos  and  in  part  to  its  anaesthetic  condition. 

An  interstitiaikeratitis  is  also  at  times  seen.  - 

Two  forms  of  iritis  are  described :  one  a  plastic  iritis  having 
no  characteristic  features,  the  other  resulting  in  the  formation 
of  a  leproma  at  the  base  of  the  iris. 

Infection  of  the  ciliary  body  and  anterior  part  of  the  retina 
and  choroid  is  common,  the  posterior  segment  of  the  globe  in 
large  measure  escaping. 

It  is  this  involvement  of  the  deeper  coats  of  the  eye  which  is 
chiefly  responsible  for  the  blindness  which  so  often  ensues. 

Leprosy  is  a  rare  disease  in  England,  but  cases  with  eye 
complications  are  reported  by  Treacher  Collins  2  and  Rockliffe.3 

1  La  Clinica  oculistica,  1909,  3437. 

2  Trans.  Opk.  Soc.  U.K.,  1909,  xxix...  223. 

3  Trans.  Oph.  Soc.  U.K.,  1902,  xxii.,  151. 


CHAPTER   VI 

Leber's  disease — Amaurotic  family  idiocy  (Tay-Sach's  disease) — Con- 
genital word  blindness — Night  blindness — Blue  sclerotics — Ocular 
torticollis — Jaw  blinking — Cyclic  dilatation  of  the  pupil — Abnor- 
malities of  the  retinal  vessels — Medullated  nerve  fibres. 

LEBER'S   DISEASE. 

THIS  is  an  hereditary  disease  characterised  by  the  develop- 
ment of  partiaToptic  atrophy  and  serious  permanent  impairment 
of  central  vision,  the  peripheral  visual  fields  being  unaffected  ; 
there  is  no' associated  constitutional  disturbance. 

It  was  first  described  by  Leber  in  1 87 1 , l  who  believed  that  it 
was  more  frequent  in  neuropathic  individuals. 

Many  members  of  a  family  may  be  affected  ;  thus  Leber 
collected  fifty-five  cases  in  sixteen  families.  Norris  2  reported 
a  family  of  seven  children  all  of  whom  were  afflicted  with  the 
disease.  In  three  generations  of  the  same  family  fourteen 
individuals  were  affected  ont  of  a  total  of  twenty-two. 

It  was  shown  by  Habershon  3  that  the  disease  may  involve 
at  least  five  generations  ;  it  may,  however,  skip  a  generation,  for 
in  five  out  of  Leber's  sixteen  families  the  disease  was  inherited 
through  the  mother,  though  she  was  not  affected. 

Sex. — Females  are  not  exempt  from  the  disease,  but  are 
affected  much  less  often  than  males,  in  the  proportion  of  one 
to  nine  according  to  Leber,  though  published  cases  would  seem 
to  show  a  less  disproportion. 

Age. — Leber's  original  statement  was  that  the  disease 
started  within  a  few  years  of  puberty  between  the  ages  of 
eighteen  and  twenty -three,  and  whilst  this  is  the  commonest 
period,  many  cases  have  been  reported  well  outside  these 
limits. 

1  Archiv.  filr  Ophth.,  1871,  xvii.,  ii.,  249  ;   "  Graefe  und  Saemisch  Handbuch," 
1877,  v.,  824. 

2  Tram.  Arner.  Ophlh.  Soc.,  1880 — 84,  iii.,  673. 

3  Trail*.  Ophlh.  Soc.  U.K.,  1888,  viii.,  190. 


TENDENCY   TO  RECOVERY  215 

Thus  Sedgwick,1  so  early  as  1862  published  the  case  of  a 
man  who  became  blind  at  the  age  of  sixty  and  whose  four  sons 
lost  their  sight  at  the  ages  of  fifty-six,  forty-six,  forty-six  and 
forty -two. 

Fuchs  2  published  cases  in  three  families  whose  ages  ranged 
from  twenty -one  to  fifty-seven,  and  Norris  3  two  cases  aged 
forty-nine  and  fifty.  On  the  other  hand,  the  average  age  of 
Norris's  fourteen  patients  was  sixteen  years. 

Course  of  the  Disease. — The  onset  is  usually  rapid  though  not 
sudden,  and  whilst  botfe-eyes  are  always  affected,  they  are  but 
seldom  involved  simultaneously  ;  it  is,  however,  unusual  for  the 
interval  before  the  second  eye  becomes  affected  to  be  so  long  as 
sijTinonths.  After  the  first  symptoms  the  sight  fails  rapidly  for 
some  days  or  weeks,  and  afterwards  more  slowly  for  several 
months. 

Later  some  improvement  generally  occurs,  so  that  ultimately 
the  patient  is  usually  able  at  least  to  get  about  alone,  and  in 
some  cases  recovery  has  been  complete.  vThus  Nettleship  4 
reports  the  case  of  a  man  whose  vision  was  reduced  to  6/60  in 
five  weeks  ;  eight  months  later  improvement  began  to  s^et  in, 
until  ultimately  he  could  read  the  smallest  type  with  ease;.  A 
brother  and  two  cousins  also  had  the  disease,  and  one  of  the 
latter  also  completely  recovered.  Batten  5  reported  a  case  in 
which  the  vision  was  restored  to  6/6,  although  the  discs  were 
somewhat  pale,  and  a  number  of  other  observers,  Frost,  Johnson 
•''  Taylor,  Lawford,  have  reported  instances  of  complete  recovery 
of  sight. 

It  may  be  said  that  patients  never  go  blind  from  Leber's 
disease,  but  that  serious  permanent  impairment  of  vision  is  the 
rule. 

The  visual  defect  takes  the  form  of  a  central  scotoma  for 
white  as  well  as  colours,  which  extends  for  about  7  degrees  from  -'< 
the  fixation  point,  the  peripheral  visual  field  remaining  uncon- 
stricted  (Fig.  71). 

1  Quoted  Habershon;  Trans.  Ophth.  Soc.  U.K.,  1888,  viii.,  191. 
-  Klin.  Mortals,  f.  Augen,  1879,  xvii.,  332. 

3  Trans.  Amer.  Ophfh.  Soc.,  1880—84,  iii.,  355. 

4  Trans.  Ophth.  Soc.  U.K.,  1903,  xxiii.,  109. 

5  Trans.  Ophth.  8uc.  C  K.,  1909,  xxix.,  141. 


216 


LEBER'S  DISEASE 


In  the  early  stages  a  mild  degree  of  change  in  the  nerve  head 
occurs,  it  takes  the  form  of  obscuration  of  the  edges  of  the  disc 
without^~oWIouiswelling ;  haemorrhages  or  exudates  do  not 
occur :  there  is  no  change  to  which  the  term  papillcedema  can 
properly  be  applied. 

When  the  acute  stage  is  passed  a  mild  degree  of  optic  nerve 


FIG.  71. — To  show  the  defects  in  the  visual  fields  in  a  case  of  Leber's 
disease.  (Gordon  Holmes.)  Lined  area  =  complete  blindness  to 
a  15m.  m.  white  square.  Dotted  area  =  partial  blindness  to  the 
same  object.  The  broken  line  encloses  the  only  area  in  which  a 
15  m.m.  bluesqu  are  could  be  seen.  The  defects  in  the  right  eye 
were  similar. 

atrophy  remains,  the  disc  showing  the  characters  of  a  secondary 
atrophy. 

Fisher  1  suggests  reasons  for  thinking  that  Leber's  disease 
may  be  due  to  disturbances  or  excessive  physiological  activity 
of  the  pituitary  body. 

Thus  he  draws  attention  to  the  neuropathic  tendencies  in  the 

1  Trans.  Ophth.  Soc.  U.K.,  1916,  xxxvi.,  299. 


AM  AV  EOT  1C  FAMILY  IDIOCY  217 

two  conditions,  to  the  occurrence  of  subjective  visual  pheno- 
mena in  each,  and  to  the  fact  that  both  show  certain  connections 
with  the  sexual  functions.  The  ophthalmoscopic  appearances 
of  the  optic  discs  are  similar  both  in  the  early  and  late  stages, 
and  he  produces  some  X-ray  evidence  which  receives  support 
from  Zentmayer  l  and  Brunner  2  to  show  that  changes  in  the 
pituitary  fossa  may  be  present  in  Leber's  disease. 

In  conclusion  he  expresses  the  opinion  "  that  when  an 
opportunity  offers  to  some  observer,  it  will  be  proved  that 
Leber's  hereditary  optic  atrophy  is  primarily  due  to  an  inherited 
temporary  disease  of  the  pituitary  body.". 

AMAUROTIC   FAMILY  IDIOCY   (TAY   SACHS  DISEASE). 

The  credit  for  the  recognition  of  this  disease  is  shared  con- 
jointly by  Waren  Tay  3  and  Sachs.4 

The  condition  is  limited  to  the  children  of  Jewish  parents  and    ' 
is  invariably  fatal. 

The  slight  histological  changes  in  the  nerve  fibres,  combined 
with  the  very  characteristic  changes  in  the  nerve  cells,  are  so 
unlike  the  morbid  anatomy  of  any  other  condition  that  it  seems 
clear  that  Amaurotic  Family  Idiocy  is  a  disease  sui  generis. 

A  marked  familial  tendency  is  often  manifested,  as  in  a 
family  reported  by  Kingdon  and  Risien  Russell  5  in  which  four 
out  of  seven  children  were  affected.  The  sexes  are  about 
equally  involved. 

The  clinical  progress  of  the  disease  may  be  conveniently 
divided  into  three  stages  (Kingdon  and  Russell). 

First  Stage. — The  children  are  normal  at  birth,  the  first 
symptoms  being  noticed  at  aboutTEnree  months  of  age.  There 
is  weakness  of  the  muscles  of  the  neck  and  back,  and  the  child 
begins  to  lose  its  sight.  Around  the  yellow  spot  a  greyish - 
white  area  is  seen  which  is  about  twice  the  size  of  the  optic  disc, 
and  at  its  centre  is  a  small  dark  red  area.  These  appearances 
If  remain  unchanged  from  the  time  of  their  appearance  till  death  ; 

1  Archiv.  of  Ophth.,  1918,  xlvii.,  627. 

2  Trans.  Amer.  Ophth.  Soc.,  1912—14,  xiii.,  162. 

3  Trans.  Oph.  Soc.  U.K.,  1881,  i.,  55;   1884,  iv.,  158  ;  and  1892,  xii.,  125. 

4  Jour,  of  Nerv.  and   Mental   Dis.,  New  York,    1887,    xiv.,  544;     1892,    xvii., 
603:   New  York  Ned.  Jour.,  1896,  Ixiii.,  697. 

5  Trans.  Roy.  Med.  Chir.  Soc.  of  London,  1897,  Ixxx.,  87. 


218 


AM AU ROT  1C  FAMILY  IDIOCY 


they  are  not  present  at  birth.  Atrophy  of  the  optic  nerve 
follows  till  complete  blindness  ensues. 

During  the  Second  Stage  the  infant  is  unable  to  sit  up  owing 
to  the  increasing  muscular  weakness,  and  becomes  apathetic. 

Third  Stage. — Finally,  atrophy  of  the  muscles,  exaggeration 
of  the  deep  reflexes,  rigidy  of  the  extremities,  and  retraction  of 
the  head  are  developed.  There  is  increasing  emaciation,  and 
convulsions  may  occur.  Life  is  rarely  prolonged  beyond  two 
years.  The  hearing  is  unaffected. 

The  morbid  anatomy  of  this  disease  has  been  thoroughly 


FIG  72. — The  ventral  horn  cells  from  a  case  of  amaurotic  family  idiocy. 
The  nuclei  are  excentric  and  are  surrounded  by  the  only  stainable  sub- 
stance which  persisted.  Vacuoles  are  present  in  many  of  the  cells. 
All  the  cells  seemed  very  poor  in  dendrites,  and  the  origin  of  the  axis 
cylinders  could  hardly  be  recognised.  (Poynton,  Parsons,  and 
Gordon  Holmes.) 

investigated  by  a  number  of  workers,  and  it  has  been  shown 
that  the  essential  primary  changes  are  limited  to  the  ganglion 
cells^throughout  4herl3o3y,  foot  only  in  the  retina  and  central 
nervous  system,  but  also  in  the  sympathetic  ganglia.' 

It  was  demonstrated  by  Poynton,  Parsons  and  Holmes  J 
that  there  may  be  marked  disease  of  ganglion  cells,  whilst  the 
corresponding  nerve  fibres  are  apparently  unaffected,  and  that 
the  interfibrillary  protoplasm  of  the  nerve  cells  seems  to  be 
more  affected  than  the  neurofibrils ;  that,  indeed,  these  latter 
1  Brain,  1906,  xxix.,  180. 


HISTOLOGICAL   CHANGES 


219 


are  only  afterwards  destroyed,  as  a  consequence  of  the  disease 
of  the  inter-fibrillary  protoplasm  (Fig.  72). 

As  the  cells  become  destroyed,  a  marked  secondary  neuroglial 
proliferation  takes  place,  and  it  is  as  a  consequence  of  this 
increase  that  the  brain,  as  pointed  out  by  Mott  and  Carlyll l 
is  increased  rather  than  diminished  in  bulk. 

The  retinal  changes  have  attracted  the  special  attention  of 
Verhoeff,2  Hancock  and  Coats,3  Poynton,  Parsons,  and  Holmes,4 
and  Cohen  and  Dixon,  all  of  whom  have  taken  particular  care 


FIG.  73.— A  section  through  a  part  of  the  spinal  cord  treated  by  Weigert  s 
stain  to  show  the  increase  of  the  neuroglia.  The  sclerosis  of  the 
pyramidal  tract  as  shown  by  the  darker  tinting,  contrasts  with  the 
pallor  of  the  neighbouring  spino-cerebellar  tracts.  (Poynton, 
1 'arsons,  and  Gordon  Holmes.) 

to  remove  and  fix  the  eyes  within  a  very  short  time  of  death. 
These  observers  are  agreed  in  every  essential  concerning  the 
changes  present.  These  changes  are  absolutely  confined  to  the 
ganglion  cells  and  their  processes,  and  consist  in  the  entire 
absence  of  normal  Nissl  granules  and  destruction  of  the  normal 
cell  processes  (Fig.  7 2) .  Thus  Verhoeff  says :  €with  the  exception 
of  the  ganglion  cells  the  retina  is  absolutely  normal^  not  a  single 

1  Proc.  Roy.  floe.  Med..  1911,  iv.,  Path.  Sect.,  147,  andUrcAtv.  of  Neurology, 
1907.  iii.,  218. 

2  Archiv.  of  Oph.,  1909,  xxxviii.,  107. 

3  Brain,  1911,  xxxiii.,  514. 

4  Brain,  1906,  xxix.,  180. 


220  CONGENITAL    WORD   BLINDNESS 

normal  ganglion  cell  is  anywhere  to  be  seen  "  ;  and  Coats  "  Not 
a  single  cell  is  to  be  found  in  which  they  (the  Nissl  granules) 
are  normal."  The  nuclei  are  shrunken,  there  is  no  retinal 
oedema  (Verhoeff). 

Mott  showed  that  these  changes  were  present  in  the  brain, 
cerebro  -  spinal  ganglion  cells,  and  also  in  the  sympathetic 
system  ;  Sachs  showed  they  were  present  in  the  pyramidal 
cells  of  the  cortex. 

With  regard  to  the  changes  which  produce  a  cherry -red  spot 
at  the  macula  surrounded  by  an  area  of  milky  haze,  Coats 

Fsays  it  is  clear  in  this  disease  that  changes  in  the  protoplasm 
of  the  ganglion  cells  is  capable  of  producing  this  appearance. 
He  uses  this  observation  to  support  Elsclmig's  contention, 
that  the  similar  ophthalmoscopic  appearances  seen  in  cases  of 
embolism  of  the  central  retinal  artery  are  not  due  to  retinal 
oedema,  but  are  due  to  coagulation" necrosis  of  the  ganglion  cells 
from  cutting  off  of  their  blood  supply  ;  there  is,  however,  this 
very  marked  difference  between  the  two  conditions,  that  in 


the  one  the  appearances  persist  till  death,  whereas  in  the  case 
of  embolism  of  the  retinal  artery  they  completely  disappear 
within  about  a  fortnight. 

CONGENITAL   WORD   BLINDNESS. 

The  first  cases  of  this  condition  published  in  English  literature 
were  by  Hinshelwood  l  who  later  incorporated  what  was  known 
of  the  condition  in  his  book  on  the  subject. 

In  congenital  word  blindness  there  is  great  difficulty  in 
reading  the  written  word,  so  that  reading  and  spelling  are 
exceedingly  irksome  or  impossible,  whilst  other  objects  are 
recognised  quite  well.  The  auditory  memory  for  words  and 
numbers  is  unaffected,  numerals  give  rise  to  much  less  trouble 
and  the  children  are  often  good  at  arithmetic.  The  subjects 
of  the  condition  are  quite  bright  and  intelligent,  often  exhibiting 
a  marked  mechanical  bent. 

So  soon  as  they  go  to  school  it  is  noticed  that  they  do  not 
learn  to  read  or  copy,  and  have  difficulty  with  the  alphabet, 

1  Lancet,  1900,  i.,  1506;  Brit.  Med.  J .,  1904,  ii.,  1303;  Oph.  Review,  1902, 
xxi.,  91. 


CLINICAL   MANIFESTATIONS  221 

and,  in  consequence,  they  are  apt  to  be  blamed  for  unwillingness 
or  stupidity,  although  in  other  respects  they  are  bright  and  equal 
to  the  other  children.  They  never  read  books  for  pleasure. 

Anyaegree  of  the  defect  may  occur,  from  a  condition  in 
which  there  is  more  than  the  usual  difficulty  experienced  in 
earning  to  read,  to  cases  in  which  it  is  impossible  to  do  so. 

Hinshelwood  says  of  one  of  his  cases  that  while  he  could 
recognise  a  few  letters  of  the  alphabet,  he  had  almost  no  visual 
memory.  Nettleship  l  says  of  a  case  :  "  He  receives  information 
with  ease  if  conveyed  orally,  reading  is  exceedingly  difficult  to 
him,  yet  he  can  pronounce  words  spelt  out  to  him.  Immense 
pains  have  been  taken  in  trying  to  teach  him  to  read." 

Some  children,  after  much  labour  and  great  care  expended  on 
them,  have  overcome  their  defect  to  a  very  marked  extent. 
Thus  Nettleship  says  of  one  of  his  cases  :  "After  an  enormous 
amount  of  patience  and  perseverance  this  boy  learned  to  read 
easily,  and  he  now  reads  both  to  himself  and  aloud  with  facility 
and  pleasure :  he  is  now  a  lawyer." 

With  others  the  defect  remains  a  serious  handicap  throughout 
life. 

Fisher  2  suggests  that  there  are  two^groups  of  cases.  In  the 
(jone  there  is  a  congenital  failure  of  development  of  the  visual 
memory  centre  in  the  left  angular  and  supra-marginal  gyrus^ 
and  in  the  second  group  this  same  centre  is  damaged  during 
birth  by  a  limited  meningeal  haemorrhage,  and  so  corresponds 
with  the  spastic  palsies  of  children.  Bastian  ascribes  it  to  a 
commissural  "defect. 

Incidence  and  Frequency. — A  number  of  these  children  are 
brought  to  "Hospital  because  it  is  assumed  that  they  cannofsee 
to  read ;  examination,  however,  fails  to  reveal  any  abnormality 
in  the  eyes,  and  in  the  rush  of  hospital  work  some,  no  doubt, 
fail  to  get  recognised. 

Thomas  3  calculates  that  one  in  1,000  children  in  elementary 
schools  suffer  from  the  condition. 

It  is  of  much  greater  frequency  in  boys  than  hi  girls.     Thus. 

1  Ophth.  Review,  1901,  xx.,  61. 

2  Trans.  Ophth.  Soc.  U.K.,  1910,  xxx.,  216. 
8  Public  Health,  May,  1908,  xxi.,  90; 


222  CONGENITAL  NIGHT  BLINDNESS 

in  100  cases  collected  by  Thomas  in  the  London  County  Council 
special  schools  seventy-six  were  boys.  He  estimates  that  it  is 
five  or  six  times  more  common  in  boys  than  in  girls,  and  other 
authors  are  in  general  agreement  with  these  figures. 

NIGHT   BLINDNESS. 

The  facility  with  which  normal  individuals  see  in  dim  illu- 
mination varies  within  ratherwi3e^-ttmits,  and  those  with 
myopia  of  any  considerable  degree  are  usually  especially 
defective  in  this  respect. 

There  are,  however,  several  sharply  defined  groups  of  cases 
in  which  patients  are  unable  to  see  so  soon  as  it  becomes  dusk  ; 
some  are  due  to  acquired  disease,  some  are  hereditary  and 
congenital,  with  or  without  discoverable  disease  of  the  eye. 

Congenital  Night  Blindness. — This  is  a  condition  in  which 
inability  to  see  at  night  is  the  only  symptom.  The  fundi  are 
normal,  the  visual  fields  are  complete,  the  visual  acuity  by  day 
is  unaffected  ;  it  is  unassociated  with  any  physical  defect,  is 
stationary,  and  does  not  lead  to  blindness,  and  both  sexes  are 
about  equally  affected. 

During  the  war  many  men  whose  lives  had  been  spent  in 
and  around  towns  and  cities,  where  real  "darkness  is  seldom, 
if  ever,  experienced,  found  that  at  night  they  had  to  be  led 
by  their  companions  over  duck  boards  or  shell-broken  ground, 
and  thus  were  first  made  aware  of  their  comparative,  though 
perhaps,  slight  defect. 

Nettleship  has  traced  this  condition  through  nine  genera- 
tions l ;  he  gives  a  genealogical  tree  commencing  in  the  year 
1637,  comprising  2,121  individuals,  and  of  these,  seventy-two 
males  and  sixty-two  females  are  known  to  have  suffered  from 
night  blindness.  Other  cases  are  reported  by  Nettleship,2 
Leber,3  and  Sinclair.4 

Night  blindness  is  a  prominent  feature  of  two  hereditary 
diseases  of  the  retina,  retinitis  pigmentosa  and  retinitis  punctata 

1  Trans.  Ophth.  8oc.  U.K.,  1907,  xxvii.,  269. 

2  Boy.  Lond.  Ophth.  Hosp.  Reports,  1887,  xi.,  68. 

3  Grctf.fe  und  Saemisch  Handbuch,  1877,  v.,  648. 

4  Ophth.  Review,  1905,  xxiv.,  255. 


ACQUIRED   NIGHT  BLINDNESS  223 

albescens.  In  each  of  these  conditions  there  are  very  character- 
istic retinal  changes,  usually  associated  with  changes  in  the  lens, 
vitreous,  and  choroid ;  the  visual  defect  is  severe  and  pro- 
gressive, and  virtual  blindness  ultimately  ensues.  £** 

Acquired  Nyctalopia. — In  extensive  diffuse  inflammatory 
disease  of  the  retina  and  choroid,  e.g.,  syphilitic  chorio-retinitis, 
nyctalopia  is  usually  present. 

Nyctalopia  associated  with  Xerosis  of  the  Conjunctiva. — There 
is  a  form  of  temporary  acquired  night  blindness  which  is 
associated  with  the  presence  of  small  xerotic  patches  on  the 
conjunctiva.  These  patches  are  triangular  in  shape  with  their 
base  adjacent  to  the  corneal  margin,  and  are  limited  to  that 
part  of  the  conjunctiva  which  is  exposed  when  the  eyes  are  open. 

In  some  cases  the  general  nutrition  is  defective ;  thus  Schiele  1 
points  out  its  prevalence  in  Kursk  after  the  LenteiTfasts.  In 
other  cases  the  subjects  appear  to  be  in  perfect  health. 

Hepburn  2  states  that  it  is  most  frequent  in  the  summer 
months,  and  that  90  per  cent,  of  his  patients  were  males  ;  he  says 
that  if  the  xerosis  patches  disappear,  so  also  does  the  visual 
defect. 

Ishihara  3  found  the  fat  contents  of  the  blood  deficient  and 
so  indicated  the  line  of  treatment ;  he  states  that  oil  obtained 
from  the  flesh  of  eels  is  a  popular  remedy  in  Japan,  and  is 
especially  beneficent. 

Treitel  4  has  shown  that  if  the  patient  is  kept  in  the  dark, 
the  night  blindness  is  recovered  from  in  the  course  of  a  day 
or  two. 

BLUE   SCLEROTICS. 

Under  this  somewhat  misleading  title  an  interesting  form  of 
hereditary  disease  has  been  described,  in  which  the  sclerotic 
coat  of  the  eye  is  so  thin,  that  the  underlying  pigmented  coat 
shows  through  and  produces  a  deep  blue  colour  ;  in  addition, 
in  a  considerable  proportion  of  cases,  the  bones  are  fragile, 

1  Wochenschr.  fur  Therapie  und  Hygiene  des  Auges,  1904,  32. 
-  Trans.  Ophth.  Soc.  U.K.,  1910,  xxx.,  167. 

3  Klin.  Monats.f.  Aug.,  1913,  xv.,  596. 

4  von  Graefe's  Archiv.f.  Op,,th.,  1885,  xxxi.,  139. 


224  BLUE  SCLEROTICS 

t'  I/ 

and  the  ligamentous  structures  about  the  joints  so  weak,  that 

fractures  and  sprains  occur  from  very  slight  violence. 

The  incidence  is  about  equal  in  the  two  sexes  and  the  trans- 
mission is  alwayscfirect ;  thus,  in  a  family  of  whom  some  are 
affected,  the  children  of  those  with  white  sclerotics  are  never 
affected,  whilst  those  with  blue  sclerotics  may  or  may  not 
transmit  the  disease.1 

It  has  been  shown  2  that  those  affected  are  usually  of  small 
stature  as  compared  with  the  unaffected  members  of  the  family. 

The  fractures  mend  without  trouble,  are  not  associated  with 
malignant  disease,  and  the  condition  does  not  appear  to  shorten 
life.  Several  extensive  pedigrees  have  been  reported,  but  it 
is  notable  that  in  some  there  is  no  mention  of  fragility  of  the 
bones. 

Burrows  published  one  composed  of  twenty-nine  indi- 
viduals, of  whom  thirteen  had  blue  sclerotics,  and  of  these 
nine  suffered  from  brittle  bones,  multiple  fractures  occurring 
in  seven  of  them  ;  whilst  of  fourteen  with  white  sclerotics  of 
whom  he  had  reliable  information,  not  a  single  one  was  known 
to  have  sustained  a  fracture. 

Conlon's  3  similar  group  extended  to  five  generations. 

Stephenson  4  published  a  pedigree  of  twenty-one  individuals, 
which  was  apparently  afterwards  extended  by  Harman 5  to 
five  generations  and  fifty-five  individuals  ;  of  these,  thirty -one 
had  blue  sclerotics,  but  in  none  of  these  is  any  mention  made  of 
fractures  ;  in  several  cases  the  presence  of  embryontoxoji  at 
the  corneal  margin  is  mentioned. 

The  association  of  the  two  conditions  appears  first  to  have  been 

recognised  by  Eddowes  6  in  a  boy  whom  he  treated  for  nine 

independent  fractures  in  the  course  of  two  years.     He  says  : 

'  This  child  had  the  darkest  sclerotics  which  I  ever  remember 

to  have  seen."     He  speaks  of  another  patient,  a  girl,  who  had 

1  Burrows,  Brit.  Med.  Jour.,  1911,  ii.,  16. 

2  Cockayne,  Ophthalmoscope.,  1914,  xii.  217,  and  Proc.  Roy.  Soc.  Med.,  1914,  vii., 
part  i.,  Sect.  Dis.  of  Child.,  101 ;  Harman,  "  Ophthalmoscope,"  1910,  viii.,  560. 

3  Boston  Med.  and  Surg.  .1 .,  1913,  clxix.,  16. 

4  Ophthalmoscope,  1910,  viii.,  330. 

5  Ophthalmoscope,  1910,  viii.,  559. 
8  Brit.  Med.  Jour.,  1900,  ii.,  222. 


FRAGILE   BONES  225 

sustained  several  fractures,  on  one  occasion  by  falling  on  a  bed  ; 
her  father  had  broken  his  arm  whilst  putting  his  coat  on. 

Adam  l  reports  a  case  in  which  ten  fractures  had  occurred. 

Pathology. — It  seems  that  in  these  individuals  there  is  a 
defect,  not  only  in  the  fibrous  coat  of  the  eye,  but  frequently 
also  in  this  constituent  of  the  bones  and  of  the  ligaments  and 
capsules  of  the  joints.  Eddowes  says  :  "  I  would  suggest  that 
the  transparency  of  the  sclerotics  indicates  a  want  of  quality  or 
quantity  of  the  fibrous  tissue  forming  the  framework  of  the 
various  organs  of  the  body,  and  probably  explains  the  want  of 
spring  or  toughness  in  the  bones  of  these  peculiar  individuals." 

Buchanan2  was  fortunate  enough  to  obtain  an  eye  for 
examination  which  had  been  seriously  damaged  as  the  result 
of  an  accident.  The  sclerotic  was  bright  blue  in  colour  ;  there 
is  no  mention  of  fractures.  The  sclerotic  was  about  one-third 
of  the  normal  thickness,  the  cornea,  too,  was  much  thinner  than 
usual,  and  its  anterior  elastic  lamina  was  entirely  absent.  The 
fibres  of  the  sclerotic  are  described  as  being  of  normal  thickness 
but  very  deficient  in  numbers,  the  blue  colour  was  due  to  the 
pigment  layer  showing  through  the  very  thin  sclera,  and  corre- 
sponded with  the  blueness  of  a  staphyloma  in  this  region. 

Cockayne  states  that  in  his  patient  of  one  year  and  ten 
months  old,  the  compact  bone  of  the  shaft  was  normal  to 
X-ray  examination,  but  that  towards  the  extremities  the  bone 
seemed  to  be  more  transparent  than  is  normal  at  this  age  :  the 
epiphyses  showed  no  signs  of  rickets. 

OCULAR   TORTICOLLIS. 

This  term  was  applied  by  Cuignet  in  1874  to  a  compensatory 
tilting  of  the  head,  in  cases  of  paralysis  of  one  of  the  muscles 
which  control  the  vertical  movements  of  the  eyes,  and  is 
unconsciously  assumed  to  avoid  ~dfplopia. 

It  is  in  all  ways  comparable  to  the  habit  of  turning  the  head 
to  one  side,  which  is  usually  assumed  by  patients  with  a  para- 
lysed external  rectus  muscle,  the  head  being  turned  towards 

1  Berlin  OpJtth.  Gesells,,  1913. 

2  Trans.  Ophth.  Soc.  U.K.,  1903,  xxiii.,  267. 

15 


M.O 


220  OCULAR   TORTICOLLIS 

the  side  of  the  muscle  which  is  paralysed  or  paretic,  in  ordei 
to  obviate  double  vision. 

In  almost  all  the  cases  reported,  the  defect  of  movement  has 
arisen  quite  early  in  life  and  is  usually  of  congenital  origin  ; 
the  position,  however,  has  been  assumed  in  a  few  acquired 
cases,  where  the  vertical  movements  have  been  disturbed  as  a 
result  of  operative  interference,  usually  in  connection  with  the 
frontal  sinus. 

It  is  probable  that  in  a  case  of  complete  uncomplicated 
paralysis  of  the  requisite  muscles,  an  occurrence  of  great 


FIG.  74. — A  case  of  "ocular  torticollis"  showing  the  head  held  in  the 
compensated  position,  and  the  vertical  deviation  of  the  eyes  when 
the  head  is  held  erect.  (Stephenson.) 

rarity,  the  position  of  the  head  would  have  to  be  so  exaggerated 
in  order  to  compensate  for  it  that  it  would  not  be  adopted. 

The  condition  has  to  be  distinguished  from  torticollis  due  to 
contraction  of  the  sterno-cleido-mastoid,  for  which  it  has  been 
mistaken,  and  for  which  ineffective  operative  procedures  have 
,  been  undertaken.1  The  differemiia£ion  is  not  difficult  if  the 
possibility  is  borne  in  mind.  The-  sterno-mastoid  is  not  con- 
tracted and  does  not  stand  out  as  a  tight  band  under  the  skin  ; 
the  position  of  the  head  is  a  pure  tilting  to  one  side — at  an 
angle  of  30  degrees  from  the  vertical  in  Stephenson's  case  ;  the 
chin  is  not  rotated  towards  the  opposite  shoulder,  as  is  seen  in 

1  Van  der  Brugh,  Xed,  Tijfa.  v.  Geneesk.,  1905,  ii.,  365  and  606, 


JAW   BLINKING  227 

v* 

contracture  cases  ;  the  head  is  easily  bent  over  towards  the 
opposite  side  and  held  there  by  the  patient  without  effort  or 
confusion  if  one  eye  be  kept  closed ;  hemiatrophy  of  the  face, 
which  is  present  and  may  be  very  marked  in  true  torticollis,  is, 
at  most,  slight  in  ocular  torticollis :  1  in  Cockayne's  case  2  the 
asymmetry,  however,  is  said  to  have  been  marked. 

In  the  assumed  position  in  the  ocular  cases  no  squint  is 
visible,  and  diplopia  is  not  complained  of  ;  if,  however,  the 
position  of  the  head  be  corrected,  a  vertical  squint  at  once 
appears  and  double  vision  is  developed. 

The  condition  is  usually  rectified  by  a  shortening  or  advance- 
ment of  the  muscle  which  is  defective. 

Cases  are  reported  by  Dallwig,3  Landolt,4  Waclsworth,5 
llisely,8  Nicden,7  dc  Lapersonnc,8  and  others. 

JAW   BLINKING. 

This  term  is  applied  to  a  curious  phenomenon  first  described 
by  Gunn,9  in  which  a  unilateral  congenital  ptosis  is  associated 
with  an  ability  to  raise  the  upper  lid  only  when  the  mouth  is 
simultaneously  opened. 

The  recorded  cases  are  almost  all  in  infants  or  children,  and 
the  left  side  is  more  frequently  involved,  in  the  proportion  of 
18  to  7  (Sinclair).10 

As  a  rule  ptosis  is  present,  though  this  is  not  invariably  so. 
Sinclair  found  it  in  twenty-seven  out  of  thirty-two  reported 
cases.  The  ptosis  may  be  partial  or  complete,  arid  there  is 
no  voluntary  power  of  lifting  the  lid  except  to  a  small  extent 
by  the  frontalis.  On  opening  the  mouth  the  lid  is  raised,  and 
on  throwing  the  jaw  towards  the  opposite  side  by  means  of 
the  action  of  the  external  pterygoid  a  further  upward  move- 
ment is  produced. 

1  Stephenson.  Ophthalmoscope,  1913,  xi.,  113. 

-  Pro.  Roy.  Soc.  Mfd.,  1914,  vii.,  part  i.,  Sec.  for  Dis.  of  Child.,  99. 

3  Archiv.  of  Ophth.,  1901,  xxx.,  (545. 

4  Bull,  med.,  1890,  573. 

5  Trans.  Amer.  Ophth.  Soc.,  1889,  v.,  £81. 

6  Trans.  Amer.  Ophth.  Soc.,  1889,  v.,  384. 

7  Centralb.  f.  prakt.  Augen.,  1892,  xvi.,  321. 

8  Archiv.  tCophlal.,  1905,  xxv.,  585. 

9  Trans.  Ophth.  Soc.  U.K.,  1883,  iii.,  S83. 
10  Oph.  Review,  1895,  xiv.,  312. 

15—2 


228  RHYTHMIC  PUPIL   CONTRACTIONS 

A  number  of  cases  are  reported  in  which  the  phenomenon 
was  conspicuous  in  infants  when  suckling,  each  movement  of 
suckling  being  accompanied  by  a  synchronous  movement  in  the 
drooped  eyelid. 

Harman  l  has  drawn  an  interesting  parallel  with  the  asso- 
ciated movements  of  opening  the  mouth  and  dilatation  of  the 
spiracle  in  fishes.  According  to  him  the  musculature  of  the 
spiracle  is  twofold  :  a  deep  part,  corresponding  with  the  external 
pterygoid  in  man,  opening  the  mouth,  and  a  superficial  part  for 
altering  the  size  of  the  orifice  of  the  spiracle,  with  which  part 
the  facial  muscles  of  man  correspond.  Contraction  of  the 
deeper  muscles  which  open  the  jaw  (external  pterygoid)  is 
associated  with  relaxation  of  the  superficial  muscles  which 
ruard  the  orifice  of  the  spiracle  (orbicularis  palpebrarum). 

How  often  one  sees  a  patient,  when  suffering  from 
photophobia  from  any  cause,  open  the  mouth  widely  in  a  futile 
attempt  to  open  the  eyes  ! 

CYCLIC  CONTRACTION  AND  DILATATION  OF  THE  PUPIL 
ASSOCIATED   WITH   THIRD   NERVE   PARESIS. 

Cases  are  occasionally  seen  in  which  a  persisting  contraction 
and  dilatation  of  one  pupil  having  a  regular  cycle,  is  associated 
with  a  congenital  or  early  acquired  paralysis  or  paresis  of  the 
third  nerve. 

The  pupil  contracts  to  about  2  mm.,  and  so  remains  for  from 
five  to  ten  seconds  ;  it  then  dilates  more  slowly  to  a  diameter 
of  6  or  7  mm.  ;  and  again,  after  some  fifteen  or  twenty  seconds, 
a  more  rapid  contraction  sets  in,  and  this  regular  cycle  is  a 
permanent  phenomenon. 

There  is  an  intimate  relationship  between  the  constriction 
of  the  pupil  and  contraction  of  the  internal  rectus  and  between 
dilatation  and  contraction  of  the  external  rectus,  for  if  either 
of  these  muscles  is  put  into  action  the  corresponding  action 
of  the  pupil  is  hastened  ;  in  some  cases  there  is  a  similar  relation 
between  contraction  of  the  levator  palpebree  and  the  pupil. 

1  Tram.  Ophlh.  8oc.  U.K.,  1903,  xxiii.,  370. 


ANEURYSMAL  DILATATIONS 


229 


D 


FI«.  75. — Different  types  of  aneurysmal  dilatation  of  the  retinal  vessels. 
A.  Fusiform  dilatations  in  the  course  of  an  artery  :  extensive  opaque 
nerve  fibres  were  also  present.  (Pringle,  Brit.  Jour,  of  Ophth., 
vol. i.,  No.  2,  p.  88.)  B.  An arterio-venous communication.  Notethat 
whilst  the  main  artery  and  vein  are  greatly  enlarged  their  collateral 
branches  are  of  normal  size.  Five  such  communications  were 
present  in  the  patient's  two  eyes.  (Foster  Moore,  Trans.  Ophth. 
•S'or.,  vol.  xxxii.,  p.  77.)  C.  Sacculated  aneurysm  of  an  artery. 
D.  Sacculated  aneurysm  of  a  vein.  The  two  last  are  from  the  same 
patient,  and  underwent  changes  whilst  under  observation.  (Story 
and  Benson,  Trans.  Ophth.  Soc.,  vol.  iii.,  p.  108.) 

During  contraction  the  pupil  is  immobile  to  light  stimulus, 
but  is  active  to  this  stimulus  both  directly  and  consensually 
during  dilatation.1 

1  Coats,  Trans.  Ophlh.  Soc,.  U.K.,  1914,  xxxiv.   2(>2. 


230  ANEURYSMAL  DILATATIONS 

The  pupil   movements  are  accompanied   by   corresponding 
spasm  and  relaxation  of  the  ciliary  muscle.1 

Other  cases  are  those  of  Greeves  2  and  v.  Hippel.3 


ABNORMALITIES    OF    THE    RETINAL    VESSELS. 

The  retinal  vessels,  of  course,  show  varying  degrees  of 
tortuosity,  but  at  times  such  tortuosity  is  so  striking  as  to 
evoke  comment,  even  though  the  condition  must  be  considered 
to  be  physiological.  Such  degree  is  seen  most  often  in 
hypermetropic  eyes,  and  involves  the  veins  much  more  frequently 
than  the  arteries,4  but  in  some  cases  both  arteries  and  veins 
are  concerned,  as  in  Benson's  case.5 

Aneurysmal  dilatations  are  at  times  seen  on  arteries,  veins, 
and  capillaries. 

Aneurysms  of  the  large  arteries  and  veins  of  the  retina  are 
quite  unrelated  as  to  their  aetiology  to  aneurysms  of  the  large 
arteries  of  the  body. 

When  they  occur  evidence  of  some  other  disease  of  the 
retina  or  choroid  is  seldom  if  ever  absent. 

They  are  probably  congenital  in  origin.  The  aneurysms 
may  be  of  the  sacculated  or  fusiform  variety  on  either  set  of 
vessels,  and  are  always  multiple  (Fig.  75). 

Arterial  aneurysms  are  reported  by  Fisher,6  Story  and 
Benson,7  Pringle,8  and  others  ;  venous  aneurysms  by 
Story,9  and  cases  of  probable  direct  communication  between 
arteries  and  veins  are  published  by  Gunn,10  Wood,11  and  Foster 
Moore.12 


1  Franke,  Klin.  Man  (its.  f.  Augen.,  1909,  viii,  582. 

2  Pro.  Roy.  ,S'oc.  Mtd.,  1912-13,  vi.,  part,  iii.,  Sect,  of  Ophth.,  23. 

3  Klin.  Monat.f.  Augen.,  1914,  Hi.,  99. 

*  Nettlcship,  Trans.  Ophth.  Hoc.  U.K..  1882,  ii.,  57  ;  Stephen  Mackenzie,  Tranf. 
Ophth.  Soc.  U.K.,  1883,  iii.,  p.  100,  and  1884,  iv..  ir>2. 

*  Trans.  Ophth.  Soc.  U.K.,  1882,  ii.,  55. 

6  Trans.  Ophth.  Soc.  U.K.,  1903,  xxiii.,  73. 

7  Trans.  Ophth.  Soc.  U.K.,  1883,  iii.,  108. 

8  Brit.  Jour,  of  Ophth.,  1917,  i.,  89. 

*  Trans.  Ophth.  Soc.  U.K.,  1883,  iii.,  108. 

10  Trans.  Ophth.  Soc.  U.K.,  1884,  iv.,  156. 

11  Tranx.  O,,/,!/,.  Noc.  U.K.,  1909,  xxix.,  lir>. 

12  Trans.  Ophth.  Soc.  U.K.,  1912,  xxxii.,  78. 


ME  DULL  AT  ED  NERVE  FIBRES  231 

MEDULLATED   NERVE   FIBRES. 

The  importance  of  medullated  nerve  fibres  in  the  retina  lies 
in  the  ophthalmoscopic  recognition  of  them  ;  in  addition, 
however,  they  have  a  few  points  of  interest. 

It  need  hardly  be  remarked,  seeing  that  the  nerve  fibre  layer 


FIG.  76. — An  unusual  case  of  opaque  nerve  fibres  which  shows  up  the 
normal  course  of  the  nerve  fibres  in  the  affected  part  of  the  retina, 
and  the  formation  of  the  horizontal  raphe.  Scotomata  were  present 
in  the  visual  field  corresponding  with  the  patches.  (B.  T.  Lang, 
Trans.  Ophth.  Soc.  U.K.,  1920,  xl.,  178.) 

of  the  retina  overlies  the  rod  and  cone  layer,  that  if  the  fibres 
of  the  former  layer  were  medullated,  the  vision  would  be  very 
imperfect,  and  it  has  been  shown  by^B.  T.  Lang  l  that  an  area 
of  relative  scotoma  can  be  mapped  out  corresponding  with  a 
patch  of  medullated  fibres.) 

1  Trans.  Ophth.  Soc.  U.K.,  1920,  xl.,  178. 


232 


ME  DULL  AT  ED  NERVE  FIBRES 


The  patches  are  not  present  at  birth,  for  Flechsig  has  shown 
that  different  tracts  of  nerve  fibres  attain  their  medullary 
sheaths  at  different  dates,  and  normally  the  optic  nerve  fibres 
attain  their  sheath  after  birth. 

It  seems  that  the  development  of  this  sheath  is  correlated 
with  the  attainment  of  full  function,  for  it  has  been  shown  that 
if  in  a  new-born  animal  one  eye  be  kept  entirely  protected  from 
light,  the  optic  nerve  fibres  on  this  side  become  myelinated  at 
a  later  date  than  in  the  eye  which  has  been  allowed  to  function, 


FIG.  77.- — A  section  through  the  optic  disc  showing  a  patch  of  myelinated 
nerve  fibres. 

and,  conversely,  the  process  of  myelination  of  all  the  nerve 
tracts  is  said  to  be  hurried  on  in  prematurely  born  children. 
/  In  cases  of  optic  atrophy  patches  of  opaque  nerve  fibres  have 
been  seen  to  disappear.1 

Ophthalmoscopic  Appearances. — The  fibres  appear  as  opaque 
white  patches  having  an  obvious  striate  texture  ;  they  are 
usually  immediately  adjacent  to  the  disc  edge,  and  extend  a 
short  distance  outward  from  it. 

Since  the  retinal  vessels  lie  in  the  nerve  fibre  layer,  they  are 
in  places  obscured  by  the  fibres.  In  a  few  cases  patches  occur, 
isolated  in  the  retina  at  some  distance  from  the  disc,  usually 
along  one  or  other  of  the  temporal  vessels  (Fig.  76). 

In  the  normal  condition,  if  the  fibres  are  traced  from  their 
course  in  the  optic  nerve  into  the  retina,  they  lose  their  myelin 

t/ 

1  Wagenmann,  v.  Graefe's  Arcliiv.  f.  OpJilh.,  1894,  xl.,  iv  ,  256. 


MEDULLATED  NERVE  FIBRES  233 

sheaths  just  before  their  passage  through  the  lamina  cribosa, 
and  contrary  to  what  might  be  expected,  this  same  condition 
in  the  main  holds  good  where  a  medullary  sheath  is  reattained 
in  the  retina. 

It  has,  however,  been  showrn  by  Usher  1  that  in  this  case  a 
very  few  fibres  have  a  continuous  medullary  sheath  through  the 
lamina  cribrosa  into  the  patch  in  the  retina  (Fig.  77). 

1  Usher,  Ophth.  Review,  1896,  xv.,  2 ;  Mayerweg,  Archiv.f.  Augen.,  1903,  xl  vi ,  122. 


CHAPTER   VII 

Oxycephaly — Leontiasis  ossea — Migraine — Recurrent  intra-oeular  hae- 
morrhage (Kales'  disease)  —  Herpes  ophthalraicus  —  Mikulicz's 
syndrome  —  Lactation  —  Mongolian  idiocy  —  Acute  amaurosis  of 
infants. 

OXYCEPHALY. 

THE  essential  features  of  this  condition  are  a  characteristic 
deformity  of  the  skull,  associated  in  most  cases  with  exoph- 
thalmos  and  some  degree  of  optic  atrophy  causing  impairment 
of  sight. 

The  skull  is  increased  in  height,  the  forehead  sloping  gradually 
upwards  to  the  vertex  which  appears  pointed,  and,  as  a 
consequence,  the  ears  seem  to  be  placed  too  low  on  the  head. 
Prematurevsynostosis  of  some  of  the  cranial  sutures,  especially 
the  sagittal  and  coronal,  seems  always  to  be  present,  and  is 
probably  the  cause  of  the  cranial  deformity. 

Careful  measurements  of  the  skulls  in  a  serits  of  cases  have 
been  made  by  Patry.1 

Of  all  the  forms  of  cranial  distortion  this  is  the  variety  which 
is  most  often  associated  with  optic  atrophy. 

The  cause  is  quite  unknown,  but  in  a  few  cases  a  familial 
and  hereditary  tendency  has  shown  itself.  All  investigators 
are  agreed  that  males  "are  much  more  commonly  affected 
than  females,  thus  Brav  2  found  that  of  eighty-five  cases  in 
the  literature  seventy  were  males.  The  intelligence  is  not 
affected,  and  it  does  not  appear  that  life  is  curtailed. 

If  the  skull  be  examined  by  X-rays,  the  bones  of  the  vault 
more  especially,  show  certain  markings  or  depressions,  which 
give  rise  to  a  coarsely -dimpled  appearance,  to  which  the  term 
"  digital  markings  "  has  been  applied  (Fig.  78).  The  sella 
turcica  stands  out  with  unusual  clearness  and  appears  to  be 
considerably  enlarged  and  displaced  backwards.3 

1  Kfceml  d'Ophtal.,  1900,   xxviii.,  158;    Annales  ffOc.ulist.,  1900,  PXXXV.,  314. 

*  Aniuilx  of  O [thth.,  1912,  xxi.,  6. 

3  Morley  Fletcher,  Quarterly  Journal  of  Med.,  1911,  iv.,  385. 


FIG.  78.- — -A  mild  case  of  oxycephaly  in  which  no  symptoms  were  present.  The  patient, 
a  woman  of  thirty,  sought  advice  for  defective  vision,  which  was  found  to  be  due 
to  lamellar  cataracts.  There  was  no  evidence  of  optic  atrophy,  and  although 
the  eyes  were  perhaps  a  little  prominent  they  would  not  of  themselves  have  evoked 
comment.  Note  the  shape  of  the  skull  and  the  digital  markings. 


OPTIC   ATROPHY  235 

'  Eye  Symptoms. — These  are  the  most  important  of  the 
disease. 

Exophthalmos  is  present  in  a  majority  of  cases,  Beaumont 
estimates  it  as  50  per  cent.  ;  *  it  may  be  extreme  in  degree,  and 
is  due  fo  the  conformation  of  the  orbits.  The  depth  of  the 
orbits  is  much  reduced,  the  outer  wall  formed  by  the  greater 
wing  of  the  sphenoid  appears  to  be  pushed  forward,  the 
orbital  axes  being  directed  very  obliquely  downwards  and 
outwards,  a  condition  which  is  also  responsible  for  the  diver- 
gence of  the  eyes  which  is  so  usually  present.  There  is 
rarely  any  impairment  of  mobility  of  the  eyes  ;  nystagmus 
is  often  present. 

Optic  Atrophy. — There  is  no   doubt   that  optic    atrophy  is 

an    exceedingly  common  complication  of    the  condition,  and 

especially  is  this  true  of  the  cases  put  on  record.     It  has, 

however,  to  be  borne  in  mind  that  few  patients  in  whom  this 

/  symptom  was  absent  would  seek  advice  at  all  (Fig.  78). 

In  by  far  the  greatest  number  of  cases  the  atrophy  is 
due  to  antecedent'  papillcedema.  ^  Thus  Enslin  2  found  thirty- 
six  cases  of  post-neuritic  atrophy  amongst  forty-two  cases  of 
atrophy,  and  Uhthoff  3  estimates  primary  atrophy  at  10  per 
cent./  It  seems  clear  that  the  papilloadema  which  is  immediately 
responsible  for  the  atrophy,  occurs  in  earlyme — Morley  Fletcher 
and  Patry  believe  before  the  age  of  five,  Uhthoff  believes  before 
seven.  The  atrophy  seldom  leads  to  complete  blindness,  but 
after  a  certain  stage  is  reached  it  makes  no  further  advance,  and 
the  vision  then  remaining  is  retained  throughout  life  ;  it  is  often 
better  than  would  have  been  anticipated  from  the  ophthal- 
moscopic  appearance  of  the  optic  disc.  Some  patients  are 
left  with  good  useful  vision  ;  thus,  Nettleship  4  records  the 
case  of  a  man  whom  he  had  seen  eighteen  years  before.  He  says 
of  him  :  "  He  is  intelligent,  earns  a  fairly  good  living  as  a  painter, 
and  that  there  is  no  further  deterioration  of  vision  since  he 
was  last  seen,"  i.e.,  eighteen  years  earlier.  Morley  Fletcher 

1  Trans.  Oph.  Soc.  U.K.,  1910,  xxx.,  44. 

2  von  Graefe's  Archiv.  f.  Opfith.,  1904,  Iviii..  151. 

3  Trunx.  ()/>lit//.  Hoc.  U.K.,  1914,  xxxiv.,  cxiii. 

4  Trann.  Oph.  Noc.  U.K.,  1905,  xxv.,  .'58:5. 


236  LEONTTASIS  OSSEA 

'says  :  "  In  adults  the  ability  to  read  is  rare,  though  few  patients 
become  actually  blind."  Uhthoff  estimates  that  7  per  cent, 
become  totally  blind. 

Pathogenesis  of  the  Optic  Atrophy. — There  is  no  satisfactory 
evidence  that  any  narrowing  of  the  optic  foramen  is  developed. 

Friedenwald  l  thinks  the  papilloedema  and  atrophy  are  the 
result  of  direct  pressure  exerted  by  the  growing  brain,  owing  to 
synostosis  of  the  sutures  of  the  vault,  comparable  to  that  of  a 
cerebral  tumour,  a  view  in  which  Morley  Fletcher  concurs. 

Uhthoff  says  :  "I  am  convinced  that  the  increased  intra- 
cranial  pressure,  with  its  destructive  effects  upon  the  optic 
nerve  and  the  visual  functions,  is  due  to  the  disproportionate 
size,  and  consequent  pressure,  exercised  by  the  growing  brain 
against  the  non-yielding  cranium." 

Patry  2  has  reported  cases  in  which  instead  of  the  horizontal 
axis  of  the  cornea  being  greater  than  the  vertical  axis  ;ix  is 
normal,  the  reverse  has  been  the  case. 

LEONTIASIS   OSSEA. 

This  disease  is  characterised  by  an  overgrowth  of  the  bones 
of  the  face  and  skull,  which  occurs  sometimes  in  a  diffuse  manner, 
but  more  often  in  the  form  of  localised  bosses  ;  its  progress 
shows  no  tendency  to  be  limited  by  the  sutures.  It  commences 
in  early  life  and  is  slowly  progressive.  Great  distortion  of  the 
physiognomy  results.  The  cause  is  unknown. 

As  the  hyperostosis  progresses  it  encroaches  on  surrounding 
structures,  such  as  the  cranial,  nasal  and  orbital  cavities,  and 
the  antrum  of  Highmore  and  frontal  sinus,  sometimes  to  the 
complete  occlusion  of  the  last  two,  and  gives  rise  to  symptoms 
corresponding  with  the  parts  involved.  The  chief  ~  ocular 
manifestations  consist  of  epiphora  from  obstruction  of  the  nasal 
ducts,  displacement  of  the  eyes,  most  often  forward  and  out- 
ward, papillcedema  where  the  cranial  cavity  is  encroached 
upon  causing  an  increase  of  intra-cranial  pressure,  and  optic 
atrophy. 

1  Amer.  Jour.  Med.  Sci.,  1893,  cv.,  529. 
-  Annales  d'Oculist.,  1900,  cxxxv.,  314. 


MIGRAINE  237 

Sattlcr  *  reports  the  case  of  a  girl  in  whom  the  first  symptoms 
were  noticed  at  the  age  of  eleven,  and  who  died  from  a  hemor- 
rhage at  the  base  of  the  brain  at  the  age  of  twenty-one.  Besides 
other  changes,  one  frontal  sinus  was  completely  occluded, 
and  the  sphenoidal  sinus  was  much  contracted.  Two  years 
before  she  died  optic  atrophy  developed,  first  in  one  eye  and 
then  in  the  other,  but  post-mortem  examination  failed  to 
reveal  any  encroachment  upon  the  optic  foramina  which 
might  account  for  this. 

For  a  complete  account  of  the  disease  a  monograph  by 
John  Green,  jun.,2  should  be  consulted. 

MIGRAINE   AND   ASSOCIATED   COMPLICATIONS. 

In  migraine  attacks  visual  phenomena  form  one  of  the,.most 
constant  and  striking  features ;  they  usually  precede  the 
headache  and  may  take  most  varied  forms.  Thus  bright 
irregular  zig-zag  lines  may  be  seen,  the  edges  of  which  are 
sometimes  brightly  coloured,  the  so-called  fortification  spectra  ; 
at  other  times  various  defects  in  the  visual  fields  are  developed  ; 
there  may  be  hemianopia  affecting  the  lateral  fields,  or  the 
upper  or  lower  parts  of  objects  may  be  obscured,  or  less  defined 
field  defects  may  occur— a  general 'contraction  of  them  is  not 
rare,  and  sometimes  a  centrarscotoma  is  complained  of  ;  there 
may  be  scintillating  lines  or  flashes  or  sparks,  or  whirling 
objects,  coloured  or  otherwise,  or  a  more  general  and  confused 
blurring  may  be  present.3  Photophobia  is  often  prominent 
(Pig.  79). 

In  a  condition  in  which  visual  phenomena  are  so  prominent 
a  symptom,  it  is  natural  that  critical  attention  should  be 
directed  to  the  ocular  mechanism,  and  whilst  nothing  but  good 
will  come  of  the  correction  of  any  real  error  of  refraction  that 
may  exist,  there  is,  I  believe,  no  direct  causal  relationship 
between  any  ocular  abnormality,  refractive  or  otherwise,  and 
migraine. 

The  fundi  are  normal  during  an  attack. 

1  Trans.  Amer.  Ophtli.  Soc.,  1900-3,   ix.,  59. 

2  Amer.  J.  of  Ophth.,  1915,  xxxii.,  293. 

3  Liveing,  "  Megrim  and  Sick  Headache,"  Churchill,  1873. 


FIG.  79.- — Nos.  1  to  4  represent  the  gradual  evolution  of  the  fortification 
spectrum  of  migraine  as  seen  in  the  dark  commencing  quite  clo>-e 
to  the  fixation  point.  Nos.  5  to  8  represent  a  similar  phenomenon 
commencing  some  distance  from  the  fixation  point.  No.  9  represents 
a  fresh  attack  beginning  whilst  No.  8  was  still  at  its  maximum  ; 
such  secondary  attacks  never  develop  fully  unless  they  occur  in  the 
other  half  of  the  visual  field.  The  small  circle  in  each  figure  represents 
the  fixation  point.  The  bastioned  outline  is  often  brightly  coloured, 
and  the  whole  figure  has  a  "  boiling  and  trembling  "  motion.  From, 
Dr.  Airey's  paper,  Phil.  Transac.,  1870,  247.) 


OPUTHALMOPLEGIC   MIGRAINE  239 

Some  individuals  have  attacks  which  are  evidently  of  the 
nature  of  migraine,  in  which  the  phenomena  are  entirely  limited 
to  the  subjective  visual  manifestations,  a  headache  only 
occasionally  following. 

It  has  often  been  supposed  that  migraine  has  affinities  with 
epilepsy  ;  it  is,  however,  very  unusual  to  elicit  a  history  of 
epilepsy  in  those  affected,  and  Hubbell l  states  that  of  1.500 
patients  with  ophthalmic  migraine  none  had  epilepsy.  Wilfred 
Harris,2  in  speaking  of  the  hemianopia  which  accompanies 
migraine,  says  that  in  many  cases  an  epileptiform  discharge 
may  originate  in  or  near  the  half  vision  centre  on  one  side,  in 
some  cases  producing  only  temporary  hemianopia,  in  others 
spreading  and  producing  a  typical  epileptiform  fit,  and  again, 
in  others  giving  rise  to  unilateral  convulsions  without  loss  of 
consciousness. 

There  can,  I  think,  be  no  doubt  that  its  incidence  is  higher 
amongst  those  of  high  intellectual  attainments  than  in  those  of 
meaner  ability. 

Lloyd 3  lays  stress  on  migraine  as  an  exciting  cause  of 
hysterical  manifestations  in  those  predisposed,  and  attributes 
some  of  the  unusual  symptoms  to  this  association. 

Ophthalmoplegic  Migraine. — This  term  was  suggested  by 
Charcot 4  for  a  defined  group  of  cases  in  which  a  palsy  of  some 
of  the  ocular  nerves,  more  especially  the  third,  at  first  temporary, 
but  sometimes  becoming  permanent  later,  is  associated  'with 
migraine. 

In  most  cases  migraine  without  the  ocular  palsy  has  already 
existed  for  a  number  of  years,  and  the  ophthalmoplegia  first 
makes  its  appearance  after  an  attack  of  unusual  severity,  and 
thereafter  occurs  in  subsequent  attacks.  It  remains  limited 
to  the  side  on  which  it  first  occurs,  and  is  seldom  if  ever  bilateral. 

The  condition  affects  the  sexes  equally,  and  usually  begins  in 
early  life. 

The  attacks  occur  at  greatly  varying  intervals  ;  sometimes  a 

1  Jour.  Amer.  Mad.  Ass.,  1908,  li.,  480. 

2  Brain,  1897,  xx.,  307. 

3  Posey  and  Spiller,  "  The  Eye  and  the  Nervous  System,    1906,  709. 
*  Jtec.'d'Ophtal.,  1890,  xii.,  623. 


240  OPHTHALMOPLEOIC  MIGRAINE 

week  only  may  intervene,  and  at  other  times  so  long  as  four 
years  or  longer.1  The  paralysis  outlasts  the  acute  symptoms, 
and  takes  from  one  to  seven  days  to  recover.  The  recovery 
is  usually  at  first  complete,  but  later  a  residue  remains  between 
the  attacks  until  ultimately  a  permanent  and  complete  paralysis 
persists.  As  a  rule  the  shorter  the  interval  between  the 
attacks  ths  more  rapid  is  the  recovery. 

The  third  nerve  is  the  one  much  most  often  implicated,  and 
whilst  all  the  external  branches  are  involved,  the  internal 
branches  often  escape. 

In  a  case  of  Karplus',  attacks  of  sickness  with  ptosis  started 
at  the  age  of  six  months,  and  occurred  about  every  two  or  three 
weeks  till  the  age  of  twenty.  After  this  age,  recovery  from  the 
paresis  between  the  attacks  was  incomplete,  and  an  external 
squint,  ptosis,  and  a  dilated  and  inactive  pupil  were  ultimately 
developed.  The  patient  died  at  the  age  of  forty-three  (vide 
infra). 

Some  observers  have  described  groups  of  cases  in  which 
recurrent  temp6rary~(3cula'r  palsies  have  been  associated  with 
severe  headaches,  which,  however,  were  not  obviously  of  the 
migrainous  type.  Thus  Holmes  Spicer  and  Ormerod  2  prefer 
the  title  "  Recurrent  Paralysis  of  Ocular  Nerves,"  and  Fisher,3 
in  describing  such  cases,  uses  the  title  "Transient  Ophthal- 
moplegia  Externa  associated  with  Attacks  of  Severe  Headaches," 
for,  he  says,  the  term  Ophthalmoplegic  Migraine  tends  to  detract 
from  a  thorough  search  for  some  organic  lesion  which  is  very 
likely  present  in  all  such  cases  ;  and  Lloyd  says  "  it  is  necessary 
for  the  present  to  hold  in  abeyance  any  theory  which  does  not 
include  the  possibility  of  an  organic  lesion."  It  is  undoubtedly 
the  case  that  a  certain  number  of  patients  who  suffer  from 
severe  recurrent  headaches  do  in  the  course  of  time  develop 
ophthalmoplegia  and  so  are  classified  under  the  title  "  ophthal- 
moplegic  migraine "  though  the  available  post-mortem 
evidence  tends  to  show  that  in  them  an  organic  lesion  is 
present. 

1  Knapp,  Boston  Med.  and  Surg.  Journal,  1894,  cxxxi.,  308. 

2  Trans.  Ophth.  Soc.  U.K.,  1896,  xvJ.,  277, 

3  Ophth.  Review,  1907,  xxvi.,  31, 


POST-MORTEM    FINDINGS  241 

Thus  of  four  autopsies,  Gubler  l  found  plastic  exudation 
surrounding  the  right  third  nerve  :  Weiss  z  says  "  the  third  nerve 
showed  a  tubercular  enlargement  at  the  point  where  it  issued 
from  the  crus  "  :  Thomsen  3  found  a  fibro-chondroma  of  the 
right  third  nerve,  and  Karplus  4  found  a  fibroma  the  size  of  a 
split  pea  in  the  course  of  the  third  nerve  connected  with  the 
dura  ;  the  tumour  was  thought  to  have  been  congenital,  and 
had  caused  degeneration  of  the  nerve  by  pressure. 

P.  C.  Knapp 5  concludes  that  the  recurrent  third  nerve 
palsy  is  due  to  some  vascular  change,  inflammatory  or 
cedematous,  in  a  focal  lesion  involving  the  root  of  the  third 
nerve,  and  that  as  the  oedema  subsides,  the  conducting  power 
of  the  nerve  is  wholly  or  partly  restored,  and  the  paralysis 
disappears. 

In  spite  of  the  foregoing,  until  satisfactory  evidence  can  be 
adduced  for  believing  that  all  such  cases  are  dependent  upon  an 
organic  lesion,  it  would  seem  useful  to  retain  the  term  "  ophthal- 
moplegic  migraine." 

Another  important  concomitant  of  migraine  is  hemianopia. 
This  has  already  been  mentioned  as  a  temporary  visua' 
phenomenon  associated  with  the  attacks,  but  a  number  of  cases 
are  recorded  in  which  the  defect,  at  first  quite  temporary,  has 
later  become  permanent,  so  that  with  some  reason  one  might 
speak  of  an  hemianopic  migraine  in  the  same  way  as  the  term 
ophthalmoplegic  migraine  is  used. 

According  to  Harris,  temporary  hemianopia  may  last  for 

'twenty -four    hours    or    more,    and   he   makes   the    important 

{  observation  that  in  all  cases  of  absolute  transient  hemianopia, 

the  dividing  line  between  the  seeing  and  blind  halves  passes 

I  through  the  fixation  point. 

Cases  in  which  permanent  hemianopia  has  resulted  have  been 
published  by  Thomas 6  (three  cases),  Gowers  7  (one  case), 

1  Quoted  by  Wadsworth,  Trans.  Amer.  Ophth.  Soc.,  1885  87,  iv.,  467. 

2  Ibid. 
s  Ibid. 

4  Wiener,  klin.  Wochensch.,  1895,  viii.,  883,  901,  922. 

5  Boston  Med.  and  Surg.  Journal,  1894,  cxxxi.,  308. 

6  Jour,  of  Xerv.  and  Ment.  Dis.,  1907,  xxxiv.,  153. 

7  Brit.  Med.  Jour.,  1909,  i.,  1403. 

10 


242  E ALES'   DISEASE 

Ormond  1  (two  cases),  Gordon  Holmes  2  (one  case)  and  Uhthoff  3 
(five  cases). 

UhthofFs  cases  all  arose  after  the  migraine  had  recurred  for 
many  years,  and  in  none  of  them  were  other  lesions  present, 
but  in  some  of  the  other  cases  weakness  of  a  limb  or  limbs  did 
occur. 

It  is  a  conspicuous  fact  that  almost  all  of  these  cases  were  in 
young  women  several  of  whom  were  pregnant. 

RECURRENT    INTRA-OCULAR    HAEMORRHAGE    OF 
UNKNOWN   CAUSE   IN   YOUNG   ADULTS. 

A  group  of  cases  of  recurrent  intra-ocular  haemorrhage 
occurring  chiefly  in  young  adult  males  associated  with  epistaxis 
and  constipation  and  with  no  other  constitutional  disease,  was 
first  defined  by  Bales  *  ami  consequently  the  condition  is  some- 
times known  as  Ealesrmsease. 

It  is  evident,  however,  on  looking  through  the  cases  reported 
under  this  head,  that  they  do  not  form  a  homogenous  group, 
and  that  cases  are  included  which  rightly  belong  elsewhere. 
Thus,  some  cases  of  Coats'  disease  are  probably  included ; 
Hiram  Woods  5  attributes  some  to  helminthiasis  ;  Weeks,  some 
to  intestinal  toxaemia,  and  some  afterwards  are  merged  into  the 
group  of  retinitis  proliferans. 

Incidence. — Bales  believed  the  condition  was  limited  to 
young  adult  males,  but  though  undoubtedly  most  frequent  in 
the  male  sex,  a  few  cases  which  appear  to  be  of  the  same 
character  have  been  reported  in  women. 

In  his  patients,  one  eye  was  chiefly  and  primarily  affected,  and 
whilst  in  most~cases  both  eyes  are  affected,  they  are  usually 
involved  in  an  unequal' degree. 

Ophthalmoscopic  Features. — The  characteristic  feature  is 
the  occurrence  of  haemorrhages  into  the  retina  which  may  or 
may  not  afterwards  invade  the  vitreous  body.  The  haemorrhages 

1  Trans.  Oph.  Soc.  U.K.,  1913,  xxxiii.,  138. 

2  Trans.  Oph.  Soc.  U.K.,  1913,  xxxiii.,  145. 
8  Trans.  Oph.  Soc.  U.K.,  1914,  xxxiv.,  226. 

4  Birmingham  Med.  Review,  1880,  ix.,  2(52  ;   and  Ophlh.  Review,  1882,   i.,  41. 
6  Trans.  Amer.  Ophth.  Soc.,  1912-14,  xiii.,  254. 


CONSTITUTIONAL   CONDITIONS  243 

are  chiefly  towards  the  periphery,  are  rather  large,  and  are 
irregular  rather  than  flame-shaped.  In  some  cases,  retinal 
exudates^iiave  in  addition  been  present.1  The  blood  may 
suddenly  burst  through  and  infiltrate  the  vitreous,  causing 
rapid  loss  of  sight  and  rendering  ophthalmoscopic  exami- 
nation impossible. 

With  the  absorption  of  the  blood  from  the  vitreous,  which  is 
a  slow  and  tedious  process,2  vision  is  recovered. 

General  Constitutional  Condition. — It  is  a  feature  of  these 
cases  that  all  evidence  of  any  serious  constitutional  disease  that 
might  be  expected  to  give  rise  to  such  haemorrhages,  is  entirely 
absent.  There  is  no  evidence  of  disease  of  the  kidneys,  heart, 
or  blood  vessels.  The  points  upon  which  Bales  laid  especial 
stress  were  the  occurrence  of  epistaxis,  constipation,  bradycardia, 
the  pulse  rate  being  habitually  below~~sixty,  dyspepsia,  head- 
aches, lassitude  and  want  of  energy  ;.  syphilis  is  not  a  cause. 
According  to^  Wilmer 3  there  is  commonly  a  delay  in  the 
coagulationume  of  the  blood. 

Eales  expresses  his  views  as  follows  :— 

"  From  the  absence  of  any  evidence  of  any  of  the  various 
blood  conditions  known  to  cause  haemorrhage  ;  from  the 
absence  of  albuminuria,  diabetes,  gout,  syphilis,  and  of  any 
high  arterial  tension  ;  from  the  character  of  the  haemorrhage, 
and  from  the  evidence  of  local  variations  of  circulation  and 
from  the  slow  pulse,  constipation,  flushing  of  the  face,  headache, 
and  puffiness  and  discoloration  of  the  eyes,  I  am  inclined  to 
attribute  this  combination  of  conditions  to  a  neurosis  affecting 
both  the  circulatory  organs  and  the  digestive  system,  leading 
on  the  one  hand  to  partial  inhibition  of  the  muscular  move- 
ments of  the  bowels,  and  to  a  vaso-motor  contraction  of  the 
vessels  of  the  alimentary  canal,  with  inhibition  of  its  secretory 
functions,  thereby  causing  dyspepsia,  constipation,  malnutri- 
tion ;  and  on  the  other  hand  to  a  compensatory  dilatation  of 
the  systemic  capillaries,  especially  those  of  the  head,  and  in 
these  cases,  of  the  retina,  causing  over-distension  of  the  venous 

1  Cunningham,  Trans.  Ophth.  Soc.  U.K.,  1912,  xxxii.,  177. 
8  Sattler,  Tram.  Amer.  Ophth.  Soc.,  1912-14,  xiii.,  250. 
3  Trans.  Amer.  Ophth.  Soc.,  1912-14,  xiii.,  251. 


244  HERPES  OPHTHALMICUS 

system  and  systemic  capillaries,  with  liability  to  rupture  on  the 
occurrence  of  any  intensifying  cause.  Hence  the  headache,  the 
epistaxis,  the  retinal  haemorrhages,  and  the  tortuosity  and 
fulness  of  the  retinal  vessels,  and  temporal  artery." 

Progress. — Recurrence  of  the  haemorrhages  is  the  rule,  their 
frequency  varying  much.  In  some  cases  the  vitreous  becomes 
permanently  opaque,  "  retinitis  proliferans  "  with  fibrous  tissue 
development  in  the  retina  occurs,  the  retina  becomes  detached, 
and  in  the  course  of  several  years  sight  is  permanently  lost.  In 
other  cases  blindness  results  from  the  onset  of  glaucoma.1 

But  not  all  cases  run  so  unfavourable  a  course  ;  in  some, 
after  a  varying  time,  the  occurrence  of  the  haemorrhages 
becomes  less  frequent  and  is  finally  arrested,  the  blood  is 
absorbed  from  the  vitreous,  and  although  the  transparency  of 
the  latter  is  not  completely  restored,  good  vision  returns. 

Sattler  2  says  useful  vision  is  often  recovered  after  a  long  and 
tedious  time.  Hiram  Woods  3  says  "  the  haemorrhages  go  on 
for  some  time,  and  then  for  some  reason  or  another  they  stop  ; 
why,  we  cannot  say." 

In  one  case  4  the  common  carotid  artery  was  tied  with  success, 
to  arrest  the  haemorrhages  in  the  right  eye,  the  left  having 
previously  been  lost  as  a  result  of  the  condition. 

I  have  seen  a  woman  in  whom  recurrences  of  the  bleeding 
showed  a  marked  tendency  to  occur  with  the  menstrual  periods, 
and  who  had  already  lost  the  sight  of  one  eye  ;  it  had,  therefore, 
been  thought  advisable  to  remove  the  ovaries,  in  the  hope  that 
the  sight  might  thus  be  preserved.  The  periods  ceased,  but 
the  retinal  haemorrhages  persisted,  and  total  blindness  resulted. 

HERPES   OPHTHALMICUS. 

When  Herpes  Zoster  attacks  the  trigeminal  nerve,  it  is  the 
ophthalmic  division  that  is  most  often  involved,  and  intra- 
ocular inflammation  is  a  common  complication.  The  condition 
is  rarely  seen  in  the  young,  and  is  more  severe  in  the  aged. 

1  Jonathan  Hutchinson,  Trans.  Ophth.  Soc.  U.K.,  1881,  i.,  26. 

2  Trans.  Amer.  Ophth.  Soc.,  1912-14,  xiii.,  250. 

3  Trans.  Amer.  Ophth.  Soc.,  1912-14,  xiii.,  2f»4. 

*  Maywcg,  quoted  by  Davis,  Trans.  Amer.  Ophth.  Soc.,  1912-14,  xiii.,  248. 


CORNEAL   CHANGES  245 

It  is  a  striking  and  interesting  fact,  that  not  only  is  involve- 
ment of  the  second  and  third  division  of  the  fifth  nerve  ex- 
ceedingly rare,  but  the  disease  often  picks  out  particular 
branches  of  the  first  division,  the  remaining  branches  escaping 
entirely.  Thus,  the  supra-trochlear  and  nasal  branches  may 
escape  or  may  alone  be  involved. 

The  eye  affection  takes  the  form  of  a  very  intractable 
keratitis  with  iritis,  and  is  said  to  occur  only  in  those  cases  in 
which  the  nasal  branch  of  the  trigeminal  is  involved,  and 
further,  to  vary  in  severity,  according  as  this  branch  manifests 
the  disease  in  a  severe  or  mild  form. 

Hutchinson  l  says  :  "  It  would,  I  think,  be  quite  safe  to 
assert  that  the  eye  scarcely  ever  suffers  much  when  the  nose 
is  not  affected,  and  that  the  severity  of  the  eruption  on  the 
one  part  is  usually  in  direct  relation  with  the  severity  of  the 
inflammation  of  the  other."  Bowman  2  says  :  "  I  have  not 
found  affection  of  the  eyeball  to  occur  specially  in  those  cases 
of  ophthalmic  zoster  in  which  the  eruption  followed  the  course 
of  the  nasal  branch.''  Hutchinson,  however,  from  a  further 
experience,  reporting  forty-one  cases  in  detail  3  says  :  "  All 
these  cases  support  my  former  inference,  that  the  nutrition  of 
the  eyeball  is  endangered  only  when  the  disease  affects  the 
oculo-nasal  twig,  a  circumstance  which  is  made  apparent  by  the 
appearance  of  the  eruption  on  the  tip  of  the  nose." 

Pain  and  tenderness  along  the  course  of  the  branches  of  the 
nerve  precede  the  eruption. 

/  It  is  a  conspicuous  fact  that  the  eye  does  not  become  inflamed 
/until  the  eruption  is  past  its  acme  and  is  already  subsiding, 
and  that  the  eye  remains  inflamed  and  irritable  for  weeks  or 
months  after  the  skin  lesion  is  soundly  healed.  The  cornea 
becomes  deeply  infiltrated  in  the  form  of  greyish  patches,  or 
lines  and  vascularisation  of  its  structure  occurs ;  occasionally 
vesicles  appear,  which  on  bursting  give  rise  to  superficial 
ulcers  ;  the  ulcers  never  perforate  and  hypopyon  does  not 
arise. 

1  Royal  London  Ophth.  Hasp.  (Moorftelds)  Reports,  1866,  v.,  191  ;  and  1869,  vi., 
181. 

2  RO^I.  Lond.  Ophth.  Hosp.  Report*,  1869.  vi.,  181. 

3  Roy.  Lond.  Ophth.  Hosp.  Reports,  1869,  vi.,  263, 


246  HERPES   OPHTHALMICUS 

When  recovery  is  complete,  there  is  usually  some  permanent 
residual  opacity  in  the  cornea  which  may  greatly  spoil  the 
sight, 

The  iritis  is  not  often  severe  and  there  is  seldom  much 
exudate. 

During  the  attack  the  sensation  of  the  cornea  is  impaired, 
and  this  impairment  lasts  for  long  after  the  disease  is  obsolete, 
and,  indeed,  it  is  probable  that  the  normal  sensitiveness  is 
never  restored,  and  from  this  cause  a  neuro-paralytic  keratitis 
may  afterwards  arise.  Apart,  however,  from  this,  recurrences 
never  occur  and  both  sides  are  never  involved. 

It  is  not  rare  to  find  associated  paralysis  of  other  cranial 
nerves,  such  as  the  oculo-motor,  abducent,  and  facial,  and  in 
the  case  of  the  facial  nerve  the  consequent  incomplete  protec- 
tion of  the  cornea,  combined  with  impaired  sensation,  is 
especially  apt  to  result  in  corneal  ulceration. 

Pathology. — The  pathology  of  herpes  zoster,  including  herpes 
ophthalmicus,  has  been  worked  out  with  especial  care  by  Head 
and  Campbell.1  They  state  that  the  first  satisfactory  report 
upon  the  post-mortem  appearances  in  herpes  zoster  was  made  by 
Sattler  2  in  a  case  of  herpes  ophthalmicus.  They  examined  the 
Gasserian  ganglion  from  a  case  in  which  death  occurred  190 
days  after  the  acute  disease  ;  it  showed  what  appeared  to  be 
the  remains  of  an  old  haemorrhage,  with  some  destruction  of 
nerve  cells  in  that  portion  corresponding  to  the  first  division 
!of  the  nerve.  The  changes  are  similar  to  those  which  occur  in  a 
posterior  spinal  root  ganglion  in  the  case  of  herpes  zoster  of  the 
I  trunk  or  limb,  and  are  summarised  as  follows.  If  the  patient 
has  died  with  the  eruption  still  out  upon  the  skin,  the  affected 
ganglion  will  be  found  to  be  in  a  condition  of  profound  inflamma- 
tion, with  areas  of  extra vasated  blood  ;  or  if  the  patient  has  died 
quite  early,  the  blood  may  be  seen  with  the  naked  eye.  Similar 
changes  are  present  in  that  part  of  the  sheath  which  covers  the 
diseased  portion  of  the  ganglion  ;  the  vessels  are  engorged, 
blood  is  extravasated,  and  the  sheath  is  invaded  by  multitudes 

1  Brain,  1900,  xxiii.,  353. 

2  Mcditin.  Jahrb.  von  cter  K.  K.  Ge.?ell<>.  der  Aerzte.,  1875,  quoted  Head  and  Camp- 
bell. 


MIKULICZ'S  SYNDROME  247 

of  small  round,  deeply  staining  cells.  As  soon  as  the  inflamma- 
tion has  expended  itself  absorption  begins,  and  ultimately  the 
focus  becomes  converted  into  fibrous  tissue ;  within  this  area 
all  ganglion  cells  and  nerve  fibres  are  destroyed  and  the  overlying 
sheath  is  thickened. 

Head  and  Campbell  comment  on  the  fact,  that  the  implica- 
tion of  a  ganglion  in  some  local  process,  such  as  malignant-"' 
disease,  can  produce  an  herpetic  eruption  which  is  indistin- 
guishable from  that  which  arises  as  a  manifestation  of  the 
acute  specific  disease. 

MIKULICZ'S   DISEASE   OR  SYNDROME. 

This  disease  was  first  described  by  Mikulicz  in  1888,  since 
when  many  cases  have  been  reported. 

The  symptom  complex  consists  in  a  chronic,  painless,  sym- 
metrical enlargement  of  certain  of  the  glands  of  the  head, 
unassociated  with  any  discoverable  general  systemic  disorder. 
In  some  cases  one  or  two  pairs  of  glands  may  alone  be 
affected,  in  others  there  may  be  enlargement  of  the  lachrymal 
glands,  the  parotid,  submaxillary,  and  sublingual  salivary 
glands,  and  even  the  glands  of  the  hard  palate  may  be  included  ; 
the  lachrymal  glands  are  usually  those  first  involved. 

The  condition  affects  the  sexes  equally,  it  occurs  in  the 
young  and  the  old,  and  except  in  so  far  as  lack  of  secretion  may 
cause  a  dry  ness  of  the  conjunctiva  or  mouth,  it  does  not  affect 
the  general  health.  Ziegler,1  whose  paper  should  be  consulted 
for  an  excellent  account  of  the  disease  and  a  full  bibliography, 
states  his  belief  that  a  careful  examination  of  all  cases  would 
probably  reveal  some  lesion  of  the  respiratory  tract  which  had 
not  been  reported. 

Mikulicz  states  that  the  enlargement  of  the  glands  is  entirely 
due  to  the  enormous  small-celled  infiltration  of  the  interstitial 
tissue  ;  he  looks  upon  it  as  a  true  Tymphoma.  Kummel  found 
the  salivary  glands  soft  and  of  marrow-like  consistence,  white, 
and  poorly  vascular.  He  found  the  gland  densely  infiltrated 
with  round  cells,  and  lymphocytes  poor  in  protoplasm. 

1  Trans.  Amer<  Ophth.  Soc.,  1910-11,  xii.,  222. 


248  LACTATION 

Thurdfield 1  who  gives  an  excellent  review  of  the  disease  and  of 
the  literature,  points  out  that  under  the  head  of  Mikulicz's 
disease  are  included  a  number  of  distinct  groups  in  which 
bilateral  swellings  of  the  salivary  glands,  either  with  or  without 
an  accompanying  enlargement  of  the  lymphatic  glands,  form 
the  most  characteristic  symptom. 

He  defines  eight  clinical  groups  of  which  the  following  are 
perhaps  the  most  important  :— 

1.  A  congenital,  hereditary,  or  family  affection. 

2.  "  Mikulicz's  disease"  proper. 

3.  "Mikulicz's  disease"  with  involvement  of  the  lym- 

phatic apparatus. 

The  progress  of  the  disease  is  very  slow ;  it  may  last  for  ten 
years  and  relapses  are  frequent  ;  it  does  not,  however,  appear 
to  affect  the  health  by  dissemination  or  otherwise. 

PROLONGED   LACTATION. 

In  a  few  cases  loss  of  sight  with  slight  ophthalmoscopic 
changes  have  been  attributed  to  prolonged  suckling. 

Two  cases  are  reported  by  Villard,2  in  which  papillcedema 
and  retinal  haemorrhages  were  present  ;  the  symptoms  started 
with  a  central  or  paracentral  scotoma.  Of  fourteen  reported 
cases  six  were  unilateral.  Three  cases  are  reported  by 
Nettleship 3 ;  the  recovery  on  weaning  was  almost  complete, 
'and  George  Derby 4  contributes  a  valuable  addition  to  our 
knowledge  of  this  subject. 

MONGOLIAN   IDIOCY. 

This  term  is  applied  to  a  form  of  imbecility  which  occurs  in 
quite  young  children  in  association  with  marked  physical 
abnormalities. 

These  abnormalities  as  they  affect  the  facev/are  responsible 
for  the  irregularity  of  contour  of  the  bony  orbits,  with  the 
obliquity^  of  the  attached  ligaments  and  tarsal  tissues,  giving 

1  Quarterly  J.  of  Med.,  1913-14,  vii.,  237. 

2  Annales  (TOculisliqne,  1912,  cxlvii.,  321. 

s  Royal  Lond.  Ophth.  Hosp.  Reports,  1893,  xiii.,  97. 
4  Archiv.  of  Ophth.,  1905,  xxxiv.,  9. 


MONGOLIAN    IDIOCY  249 

the  palpebral  fissures  their  obliquity  of  direction,  the  lids  their 
shortness  and  epicanthus,  and  the  eyes  their  apparent  faulty 
situation  ;  it  is  these  characters  which  are  responsible  for  the 
naming  of  the  type.1 

Amongst  other  physical  characters  are  dwarfism,  a  flattened 
nose,  a  protuberant  lower  lip,  a  large  tongue  the  mouth  being 
usually  kept  open,  small  and  malformed  hands,  flat  feet  and 
a  wide  gap  between  the  first  and  second  toes. 

The  children  are  mentally  defective,  but  are  of  a  happy  dis- 
position ;  they  seldom  reach  adult  age. 

Ormond  2  has  examined  the  eyes  of  forty-two  cases,  thirty- 
two  males  and  ten  females.  He  points  out  that  some  disease 
of  the  eyes  or  lids  is  exceedingly  common. 

In  over  fifty  per  cent,  of  his  cases  some  form  of  lenticular 
opacity  was  present.  In  some  the  opacity  was  of  the  lamellar 
type,  in  others  a  section  of  the  lens  was  opaque  or  the  opacity 
was  of  the  dot  variety.  The  youngest  patient  with  cataract 
was  aged  six-and-a-half  years  ;  the  oldest  was  forty-three. 
Other  abnormalities  were  : — 

Nystagmus          .          .          .      11 -9  per  cent. 
Squints       ....     21-4        ,, 
Blepharitis  or  ectropion        .     42-8        ,, 
Epicanthus          .          .          .     26-2        „ 

ACUTE    CEREBRAL    AMAUROSIS    OF    INFANCY. 

Under  the  above  title,  Gay 3  and  Nettleship  4  have  each 
published  a  series  of  cases  of  sudden  blindness  occurring  in 
infants  or  young  children,  associated  with  meningitic  symptoms, 
and  most  often  ending  in  recovery. 

Gay  says  :  "  The  eye  symptoms  in  this  series  of  cases  seem 
to  me  to  constitute  an  ophthalmological  entity,  characterised 
by  blindness,  but  unaccompanied  by  any  change  in  the  disc 
or  fundus,  and  following  upon  cephalic  symptoms  of  an  undeter- 
minate  character,  although,  as  a  rule,  suggestive  of  meningitis. 

1  Oliver,  Tram.  Amer.  Ophth.  Soc.,  1891,  vi.,  140. 

-  Trans.  Ophth.  Soc.  U.K.,  1912,  xxxii.,  69. 

3  Royal  Lord.  Ophth.  Hosp.  (Moorfelds)  Reports,  1893,  xiii.,  404. 

*  Trans.  Ophth.  Soc.  U.K.,  1884,  iv.,  245. 


250  ACUTE  CEREBRAL  AMAURO81& 

The  tendency  of  such  cases  is  usually  towards  recovery,  but 
sometimes  atrophy  of  one  or  both  discs  ensues  and  the 
blindness  becomes  permanent.  The  characteristics  are — 
sudden  onset  of  blindness,  associated  with  a  febrile  attack 
with  convulsions,  cervical  opisthotonos,  weakness  of  the  back 
and  legs,  and  general  evidence  of  meningitis,  the  pupils  are 
sluggish  and  inactive,  and  recovery  of  sight  is  usually 
complete." 

Nettleship  says  blindness  may  last  from  one  to  six  months 
before  improvement  begins,  and  recovery  takes  place  rather 
slowly  ;  on  the  other  hand,  some  cases  recover  quickly,  indeed, 
within  a  few  days.  In  a  few  cases — three  out  of  fourteen — 
permanent  blindness  with  secondary  optic  atrophy  ensues. 

Sometimes  blindness  seems  to  be  the  only  symptom.    There 

'  is  no  doubt  that  the  great  majority  are  instances  of  post-basic 

meningitis  (q.v.),  as  was  shown  by  Barlow  and  Lees,  and  it  is 

not  clear  that  any  other  condition  gives  rise  to  the  foregoing 

clinical  picture. 


CHAPTER   VIII 

Snow  blindness — Eclipse  blindness — Electric  aniblyopia — Lightning — 
Neuroparalytic  keratitis — Retro-bulbar  neuritis — Oyster  shucker's 
keratitis. 

SNOW   BLINDNESS. 

THIS  condition  is  an  affection  of  the  superficial  parts  of  the 
eye,  the  conjunctiva  and  cornea,  and  so  is  related  to  the  similar 
conditions  which  arise  in  "  electric  ophthalmia,"  and  is  quite 
distinct  from  eclipse  blindness. 

It  shows  itself  by  photophobia,  blepharospasm,  hypergemia 
and  increased  secretion  from  the  conjunctiva,  and  sometimes 
by  the  formation  of  an  erosion  or  bulla  on  the  cornea. 

The  symptoms  usually  come  on  some  hours  after  the  ex- 
posure, but  may  result  in  so  short  a  time  as  fifteen  minutes  if 
the  eyes  are  unprotected.1 

It  is  light  reflected  from  snow  which  is  chiefly  active,  and 
\Vindmark  has  shown  that  such  light  is  especially  rich  in 
ultra-violet  rays  ;  it  may  occur  on  dull,  cloudy  days  as  well  as 
in  sunny  weather. 

Daland  2  examined  3,000  Eskimos  and  showed  that  they  are 
not  immune  to  snow  blindness,  and  that  one  attack  predisposes 
to  future  attacks.  He  states  that  it  also  occurs  in  animals. 

In  addition,  Atkinson,1  from  his  South  Polar  experience, 
describes  a  temporary 'diplopia  which  persists  for  several  days 
and  completely  recovers.  This  he  attributes  to  the  tiring  of 
the  external  and  internal  ocular  muscles,  and  is  caused  by  diffi- 
culties of  vision  through  lack  of  contrast  when  the  air  is  so 
thickly  filled  with  ice  crystals  that  it  is  impossible  to  see  more 
than  a  yard  or  two  ahead. 

1  Atkinson,  Brit.  Jour,  of  Ophth.,  1921,  v.,  50. 

2  Ophth.  Sec.,  1917,  xxvi.,  116  and  Trans.  Coll.  Physic,  Philadelphia,  1916, 
xxxviii.,  370. 


252  ECLIPSE  BLINDNESS 

ECLIPSE   BLINDNESS. 

There  is  a  group  of  cases  in  which  an  over-stimulation  of  the 
retina  results  in  a  great  temporary  impairment  of  its  functions, 
and  sometimes  ends  in  a  permanent  visual  defect. 

The  best  known  and  most  frequent  of  the  causes  is  from 
looking  directly  at  the  sun,  and  as  this  form  is  most  often 
encountered  in  those  who  have  been  watching  an  eclipse,  it  is 
often  called  "  eclipse  blindness." 

Amongst  other  similar  causes  are  blindness  from  sudden 
intense  electricHashes. 

If  the  eyes  are  closed  immediately  after  viewing  the  sun, 
a  bright  after-image  is  seen,  which  rapidly  changes  in  colour 
and  soon  disappears. 

With  regard  to  this,  Mackay  l  says  :  "  The  persistence,  on 
closing  the  eye,  of  a  sun  image  which  does  not  rapidly  undergo 
alteration  in  colour,  may  perhaps  be  taken  as  the  mark  of  a 
retinal  stimulus  which  has  slightly  surpassed  physiological 
limits." 

Most  observers  agree,  that  following  the  exposure  to  the 
sun,  an  interval  of  an  hour  or  two  or  longer  elapses,  before  the 
patient  is  aware  of  a  visual  defect. 

Lawford  2  reports  a  case  in  which  the  defect  was  not  observed 
for  ten  days  ;  in  Collins'  case  there  was  progressive  deterioration 
for  a  week. 

In  all  cases  there  is  a  partial  central  scotoma  which  is  more 
complete  for  colours  ;  in  the  course  of  time  this  almost  com- 
pletely disappears  in  most  cases,  but  as  a  rule,  some  slight 
residue  will  be  found  if  the  examination  is  sufficiently  careful. 

Occasionally  a  serious  permanent  defect  remains,  thus 
the  case  of  a  patient  in  whom  the  vision  fell  to  1  /20  and 
never  recovered  beyond  1/2  is  reported  by  Dufour,3  and 
Treacher  Collins 4  reports  the  case  of  a  woman  of  forty - 
nine  who,  having  looked  at  the  sun  for  about  seven  minutes, 
was  almost  blind  in  the  left  eye  at  the  end  of  a  week  ;  seven 

1  Ophth.  Review.  1894,  xiii.,    1.  41  and  83. 

2  Trans.  Ophth.  Soc.  U.K.,  1901,  xxi.,  77. 

*  Quoted  by  Mackay. 

*  Royal  Lond.  Ophthalmic  Hospital  (Moorftdds)  Reports,  1897,  xiv.,    374. 


PROGNOSIS  253 

years  later  she  could  only  count  fingers  with  this  eye,  and  the 
appreciation  of  colour  was  much  interfered  with  ;  the  optic 
nerve  showed  partial  atrophy.  The  peripheral  visual  field  was 
complete  in  each  eye,  and  the  vision  of  the  right  eye  was  6/6. 

Metamorphopsia  is  often  a  little  troublesome  and  may  be 
permanent. 

In  a  few  cases  a  slight  disturbance  of  the  pigment  of  the 
hexagonal  pigment  layer  may  be  present,  but  any  marked 
ophthalmoscopic  change  is  quite  unusual  ;  that,  however, 
organic  changes  may  occur  is  shown  not  only  by  the  clinical 
case  referred  to,  but  also  by  Deutschman's  experiments.1 
He  focussed  a  strong  light  into  the  eyes  of  rabbits,  and  pro- 
duced not  only  ophthalmoscopic  changes,  but  also  a  limited 
area  of  disorganisation  of  the  retinal  tissues,  which  was  found 
upon  histological  examination. 

Prognosis. — The  prospect  as  to  recovery  in  all  but  the  most 
severe  cases  is  good,  and  it  is  unusual  for  the  patient  to  be 
bothered  by  any  defect  that  may  remain. 

The  time  taken  in  recovery  is  tabulated  by  Mackay  as 
follows  : — 

Group  1. — A  patient  with  V  ==  1/3  or  better  in  the  first  week 

has  a  good  chance  of  practical  recovery  in  one  month. 
Group  2. — A  patient  with  V  -=  1/3  or  better  in  the  second 

week  has  a  good  chance  of  practical  recovery  in  three 

or  four  months. 
Group  3. — A  patient  with  V  =  1/3  or  better  in  the  third 

week  has  a  good  chance  of  practical  recovery  in  five 

or  six  months. 
/Group  4. — A  patient  with  V  ==  less  than  1/3  seems  to  have  a 

bad  chance  of  recovering  V  ==  6/6. 

By  practical  recovery  is  meant  cessation  of  obtrusive  defect. 

ELECTRIC   AMBLYOPIA. 

It  is  common  to  find  a  temporary  incomplete  central  scotoma, 
and  injection  of  the  vessels  of  the  conjunctiva,  following 
exposure  to  a  momentary  intense  short  circuit  flash,  and 

1  von  Graefe's  Arch'n-.f.  Ophtli.,  1882,  xxviii.,  iii.,  241. 


254  LIGHTNING 

similar  symptoms  may  arise  in  those  who  have  to  do  with 
intense  light  of  any  kind. 

Panas  1  saw  forty-five  cases  of  eye  trouble  in  two  years 
from  short-circuit  flashing  in  the  "  Metropolitain."  The 
symptoms  were  conjunctival  injection,  superficial  burns, 
blepharospasm,  diminished  vision  and  contracted  fields.  He 
states  that  acute  forms  of  conjunctivitis,  with  photophobia 
and  pain  in  the  head,  are  common  in  large  electrical  works 
following  exposure  to  short  circuiting.  Of  his  cases  many 
recovered  in  three  or  four  weeks,  but  in  a  considerable  number 
symptoms  persisted  for  three  or  four  months. 

LIGHTNING. 

A  few  cases  are  on  record  in  which  the  eyes  have  been 
affected  by  a  lightning  flash,  in  some  of  which  organic  damage 
resulted. 

The  most  conmloTnjhange  is  the  development  of  lenticular 
opacities,  in  other  cases  striae  in  the  cornea  have  been  seen, 
and  in  a  few  optic  atrophy  has  subsequently  developed  ; 
temporary  photophobia  is  common. 

Gonin  2  reports  a  case  in  which  the  patient  was  unconscious 
for  a  quarter  of  an  hour  after  being  struck.  The  corneas  were 
hazy  for  a  few  days,  the  retinal  vessels  were  constricted,  and 
the  optic  discs  were  pale  ;  he  attributes  further  deterioration  of 
vision  to  optic  atrophy.  Radiating  opacities  were  present  in 
the  posterior  layers  of  the  left  lens,  and  twelve  months  later 
punctate  opacities  were  observed  in  the  right  lens. 

He  reviews  thirty  reported  cases,  of  which  twenty-two  had 
lens  opacities. 

Leber 3  found  nine  cases  of  cataract  amongst  eighteen 
similar  cases. 

NEUROPARALYTIC   KERATITIS. 

In  cases  where  the  functional  continuity  of  the  trigeminal 
nerve  is  interrupted,  whether  by  disease  or  as  the  result  of 
operation  for  the  relief  of  intractable  neuralgia,  the  cornea  is 
very  liable  to  become  ulcerated, 

1  Archiv.  d'Ophtal.,  1902,  xxii.,  625. 

2  Annales  d? Oculistique.,  1904,  cxxxi.,  81. 
8  Archie,  j.  Ophth.,  1882,  xxviii.,  iii.,  255. 


NEUROPARALYTIC  KERATITIS  255 

Different  views  have  been  held  as  to  the  cause  of  this  change, 
as  to  whether  it  is  due  to  exposure  and  trauma  unnoticed  by 
the  insensitive  cornea,  or  whether  it  is  due  to  the  removal 
of  some  trophic  influence.  Fuchs  l  says  :  "  If  in  a  case  of 
trigeminal  paralysis  in  which  the  eye  is  still  healthy,  we  hold 
the  lids  apart  for  one  or  two  minutes  with  the  fingers,  and  thus 
prevent  moistening  of  the  cornea,  small  depressions,  which 
soon  enlarge  and  become  confluent,  appear  in  the  epithelium  .  .  . 
a  healthy  eye  does  not  show  these  pits  at  all,  or  does  so  only 
after  a  long  time.  These  pits  prove  a  diminished  resistance  of 
the  corneal  epithelium  to  desiccation,  and  consequently  a 
disturbance  of  its  nutrition  which  was  present  before  the 
desiccation  began. 

"  Diminished  moistening  is,  therefore,  certainly  more  in- 
jurious for  such  eyes  than  for  normal  ones.  But,  as  above 
stated,  neuroparalytic  keratitis  may  develop,  even  when  the 
cornea  is  kept  permanently  covered.  Desiccation,  therefore,  is 
to  be  regarded  only  as  a  favouring  factor,  not  as  the  final  cause 
of  keratitis  neuroparalytica.  Such  a  cause  is  found  in  a 
disturbance  of  nutrition  of  the  cornea  (and  particularly  of  its 
epithelium)  produced  by  the  loss  of  innervation.  As  a  result  of 
this  disturbance  of  nutrition,  the  resisting  powers  of  the  cornea 
is  so  reduced,  that  the  latter  is  thrown  into  a  diseased  condition 
by  external  influences  which  are  so  slight,  that  a  normal  eye 
would  not  have  been  injured  by  them." 

In  the  early  stages  of  the  condition  the  corneal  epithelium 
is  desquamated  over  a  small  area ;  this  process  spreads  until 
the  whole  of  the  cornea,  except  for  a  narrow  ring-like  margin 
all  round,  appears  to  be  completely  denuded,  and  stains  a 
bright  green  if  fluorescin  be  dropped  into  the  eye. 

At  the  same  time,  the  eye  shows  comparatively  little  injec- 
tion and  pain  is  absent ;  the  whole  picture  is  distinctive  and 
unlike  any  other  condition. 

In  the  later  stages,  infiltration  of  the  substantia  propria 
becomes  more  extensive  till  it  has  a  yellow  colour,  pus  collects 
in  the  anterior  chamber,  the  cornea  perforates,  the  eye  ultimately 
being  completely  spoiled. 

1  "  Text-book  of  Ophthalmology,"  1908,  194. 


256  ACUTE   RETRO-BULBAR   NEURITIS 

A  case  of  Caisson  disease,  in  which,  among  other  symptoms, 
there  was  anaesthesia  of  all  the  branches  of  the  two  first  divisions 
of  the  fifth  nerve  with  neuroparalytic  keratitis,  is  reported  by 
Harlan.1 

ACUTE   RETRO-BULBAR   NEURITIS. 

Under  this  term  are  included  cases  of  the  local  implication 
of  the  optic  nerves,  by  toxins  or  inflammatory  processes,  which 
lead  to  great  loss  of  sight. 

Those  cases  which  are  brought  about  by  the  direct  extension 
of  an  inflammatory  process  in  the  orbit  to  the  optic  nerve  need 
not  be  considered  here,  and  the  toxic  amblyopias  are  dealt  with 
separately. 

There  is,  however,  a  group  of  cases  for  which  no  satisfactory 
cause  is  found,  which  is  characterised  by  loss  of  sight,  which 
develops  within  two  or  three  days  and  usually  affects  one  eye 
only  ;  pain  on  movement  of  the  eyeball  and  neuralgic  pains 
in  the  temple  ;  a  pupil  which  contracts  to  light,  but  the  con- 
traction of  which  is  not  maintained  ;  and  a  normal  fundus. 
The  pain  usually  precedes  the  loss  of  sight  and  does  not  last 
more  than  a  few  days.  In  many  cases  a  central  scotoma  is 
present.  It  is  usual  for  complete  recovery  to  occur,  but  in  six 
out  of  twenty  cases,  Nettleship  2  found  the  sight  was  entirely 
and  permanently  lost,  and  in  these  cases  optic  atrophy  occurred. 

No  cause  is  known,  and  consequently  such  cases  are  often 
attributed  to  gout  or  rheumatism. 

OYSTER   SHUCKER'S   KERATITIS. 

This  is  an  interesting  form  of  acute  corneal  ulcer  which 
results  from  the  impaction  of  minute  fragments  of  the  shell  of 
the  oyster  in  the  cornea.  It  is  of  frequent  occurrence  in 
Baltimore,  where  there  is  a  large  oyster  industry,  and  where  the 
edge  of  the  shell  is  chipped  off  with  a  hammer,  preparatory 
to  introducing  the  knife  to  prise  the  shell  open. 

The  condition   has   been   investigated   by   Randolph ;  3   he 

1  Trans.  Amer.  Ophth.  Soc..  1897-98,  viii.,  107. 

2  Trans.  Ophth.  Soc.  U.K.,  1884,  iv.,  186. 

8  Trans.  Amer.  Ophth.  Soc.,  1894-90,  vii.,  313. 


OYSTER  SHUCKERS  KE  RAT  IT  1 8  257 

describes  the  ulcer  as  whiter  than  other  ulcers,  having  a  sharply 
denned  edge,  and  showing  little  tendency  to  spread.  He 
showed  by  experiment  that  the  nature  of  the  ulcer  was  apparently 
due  to  the  chemical  nature  of  the  shell  fragment. 

Ulcers,  which  have  a  good  deal  in  common  with  the  above, 
are  seen  in  those  occupied  in  scraping  the  hulls  of  ships.  There 
is  a  reaction  out  of  all  proportion  to  the  size  of  the  ulcer, 
which  is  very  slow  in  healing,  and  it  is  probable  that  it  is  the 
chemical  nature  of  the  minute  fragments  which  enter  the  eye 
which  is  responsible  for  this  particular  type  of  ulcer. 


17 


CHAPTER   IX 

Tobacco — Alcohol — Methyl      alcohol — Quinine — Sodium      salicylate — 
Lead  —  Silver  nitrate  —  lodoform  —  Carbon  di-sulphide — Nitro- 
benzol  —   Aniline  —   Phenol  --    Ochronosis  --   Naphthaline   - 
Antipyrin — Santonin — Aspidium  (Filix  Mas) — The   arylarsenates 
— JEthylhydrocuprein  (Optochin). 

TOBACCO   AMBLYOPIA. 

THE  most  universal  form  of  toxic  amblyopia  is  that  which  is 
caused  by  tobacco,  whether  by  smoking,  chewing  or  snuff- 
taking,  or  as  has  been  shown  by  de  Schwenitz,  it  may  occur  in 
those  who  work  in  tobacco  factories,  even  though  they  neither 
smoke  nor  chew  tobacco. 

The  prominent  features  of  the  condition  are  impairment  of 
central  vision  with  a  central  or  para -central  scotoma  for  red 
and  green,  an  intact  peripheral  field,  complete  recovery  on 
stopping  smoking,  and  a  normal  or  nearly  normal  fundus. 

The  subjects  of  the  disease  are  often  copious  drinkers  of 
alcohol,  and  the  view  has  been  held  that  the  loss  of  vision  is 
due  to  the  combined  effects  of  the  two  noxious  agents,  conse- 
quently the  term  "alcohol-tobacco  amblyopia"  has  come  to  be 
used  by  some. 

That,  however,  the  loss  of  sight  may  result  from  tobacco 
alone  in  the  absence  of  alcohol  is  shown  by  Connor,1  who 
collected  twenty-seven  such  cases,  and  by  Lanckton  Foster.2 
Further,  the  lost  vision  may  be  restored  by  stopping  smoking, 
alcohol  being  continued  as  before. 

Jonathan  Hutchinson's  remarks  are  interesting  in  this  con- 
nection.3 He  says  :  "  Total  abstainers  from  stimulants  are 
more  liable  to  suffer  than  others,  and  although  we  sometimes 
meet  with  the  disease  in  the  intemperate,  I  have  a  strong 

1  Jour.  Amer.  Med.  Ass.,  1890,  xiv.,  217. 

2  Trans.  Amer.  Ophth.  Soc.,  1912-14,  xiii.,  516. 

3  Royal  London  Ophth.  Hosp.  Reports,  1874,  viii.,  456. 


INCIDENCE  259 

impression  that,  on  the  whole,  alcohol  counteracts  tobacco  " — 
a  view  which  finds  few  if  any  adherents. 

There  is  no  doubt  that  a  debilitated  condition  predisposes  to 
the  poisoning,  and  perhaps  this  is  especially  true  of  diabetes  ;  it 
has,  however,  to  be  borne  in  mind  that  an  amblyopia  which  is 
proper  to  this  latter  disease  may  occur  (p.  171). 

Incidence. — The  disease  for  obvious  reasons  is  much  most 
frequent  in  men  ;  it  comes  on  only  after  smoking  has  been 
continued  for  some  years  ;  it  is  uncommon  under  the  age  of 
thirty,  and  the  years  between  thirty-five  and  fifty-five  embrace 
the  greatest  number  of  cases. 

In  the  course  of  eleven  years,  amongst  12,644  ophthalmic 
out-patients,  Priestley  Smith  x  found  a  percentage  of  T20  of 
tobacco  amblyopia. 

It  is  most  frequently  brought  on  by  pipe  smoking,  and  by 
strong  shag  tobacco,  less  often  by  cigars,  and  rarely  by  cigarettes. 

Van  Millingen  2  states  that  in  Turkey,  where  smoking  is  exten- 
sively practised  both  by  men  and  women,  amblyopia  is  un- 
known, a  fact  which  he  attributes  to  the  mildness  of  the  tobacco, 
and  to  the  fact  that  it  is  almost  entirely  cigarettes  which  are 
smoked. 

The  amount  of  tobacco  which  is  necessary  for  the  production 
of  amblyopia  varies  with  the  kind  of  the  tobacco,  and  especially 
with  the  susceptibility  of  the  patient,  and  inquiry  will  often 
elicit  the  fact  that  whilst  a  very  modest  amount  is  at  present 
confessed  to,  a  month  or  six  weeks  previously  it  was  much 
larger  in  amount,  the  fact  being  that  these  folk  have  often 
independently  concluded  that  smoking  was  the  cause  of 
their  trouble. 

The  degree  of  amblyopia  is,  with  very  few  exceptions,  approxi- 
mately equal  in  the  two  eyes,  but  a  few  marked  exceptions  have 
been  reported. 

All  cases  improve  with  complete  cessation,  most  with  mere 
reduction  of  smoking.  It  may  be  safely  assumed,  either  that 
smoking  has  not  been  stopped,  or  that  the  diagnosis  is  incorrect, 
if  no  improvement  takes  place  in  three  months. 

1  "  Report  of  the  Committee  on  Colour  Vision,"  Roy.  Society,  London,  1892. 

2  Trans.  Ophth.  Soc.  U.K.,   1888,  viii.,  240. 

17—3 


260  TOBACCO  AMBLYOPIA 

It  might  well  have  been  expected  that  a  patient  who  had  once 
shown  himself  susceptible  to  the  poison  by  developing  ambly- 
opia,  would  be  likely  to  have  a  recurrence  on  resuming  the 
habit,  but  recurrences  are  seldom  seen,  and  whilst  chronic 
tobacco  poisoning  may  produce  marked  disturbances  of  diges- 
tion, of  the  heart's  action,  and  of  mental  ability,  it  is  common 
to  find  none  of  these  prominent  in  cases  of  tobacco  blindness. 

There    are    no    characteristic    or    marked    ophthalmoscopic 

/changes,  and  such  as  have  been  described  are  so  often  seen 
under  purely  physiological  conditions,  that  their  diagnostic 
value  is  almost  negligible. 

The  chief  change  described  is  a  pallor  of  the  lower  temporal 
part  of  the  disc  ;  a  few  other  quite  indefinite  changes  have  been 
described.  De  Schweinitz  states  that  "  under  rare  circum- 
stances complete  atrophy  ensues,"  and  Nettleship  l  that  "  in 
severe  cases  of  long  standing  a  general  pallor  of  it  (the  disc) 
may  occur." 

Visual  Fields. — Whilst  the  peripheral  boundaries  of  the 
visual  fields  are  normal,  there  is  a  scotoma  for  red  and  green 
•  between  the  blind  spot  and  the  point  of  fixation. 

At  first  the  scotoma  is  for  colour  only,  but  later  it  may 
become  absolute  even  to  light  perception. 

An  important  form  of  blindness  which  occurs  in  New  South 
Wales  in  Tfofses  has  been  described  by  Kendal  and  Cameron  2 
and  Barrejtt,3  which  is  believed  to  be  due  to  the  ingestion  of  the 
Australian  tobacco  plant  (Nicotiana  suaveoleus). 

On  the  other  hand,  de  Schweinitz  states  that  the  Virginia 
deer  eat  the  leaves  of  the  tobacco  plant  (Nicotiana  tabacum) 
without  detriment ;  he  suggests  that  they  have  become  immune 
to  the  drug  by  reason  of  many  years'  residence  in  the  tobacco 
district,  just  as  the  monkeys  of  certain  districts  of  the  East 
Indies  are  said  to  eat  the  seeds  of  the  Strychnos  nux-vomica 
without  harm,  whilst  to  other  tribes  of  monkeys  they  are  a 
deadly  poison. 

Pathology. — It  cannot  be  said  that  the  anatomical  site  of  the 
primary  lesion  has  been  made  certain. 

1  Trane.  Ophth.  Soc.  U.K.,  1887,  vii.,  38. 

2  Quoted  de  Schweinitz,  "Toxic  Amblyopias,"  Philadelphia,  1896,  95. 
*  Intercolonial  Med.  Jour,  of  Austral.,  1897,  ii.,  172. 


PATHOLOGY  261 

Fisher  l  argues  on  theoretical  grounds,  having  regard  to 
Langley's  work  on  the  action  of  nicotine  on  ganglion  cells  and 
their  synapses,  that  the  block  in  transmission  probably  lies 
between  the  macular  cones  and  their  ganglion  cells. 

Parsons  2  has  shown  that  there  is  no  discoverable  change  in 
the  cells  of  the  superior  cervical  ganglion  of  rabbits  as  a  result 
of  soaking  them  in  a  1  per  cent,  nicotine  solution  in  normal 
saline. 

He  suggests  as  a  tentative  working  hypothesis  3  that  the 
defect  is  due  in  part  to  : — 

(1)  Constriction  of  the  arterioles,  which  would  explain  the 

selective  choice  of  the  macular  region  in  which  the 
vascular  supply  is  sparse. 

(2)  Paralytic  action  upon  the  synapses  either  of  the  cone 

fibres  or  of  the  bipolars  or  both. 

Birch-Hirschfeld  4  examined  the  eye  of  a  man  who  had  had 
tobacco  amblyopia  for  seven  years  ;  he  describes  degeneration 
of  the  retinal  ganglion  cells  and  atrophy  of  the  nerve  fibres  with 
secondary  changes  in  the  interstitial  tissues. 

Dalen  5  obtained  the  eye  from  a  man  a  few  hours  after  death 
who  had  had  symptoms  of  amblyopia  for  nine  weeks  only  ;  he 
found  degeneration  of  the  papillo -macular  bundle. 

De  Schweinitz  6  comes  to  the  conclusion  that  the  appearances 
correspond  closely  with  those  usually  ascribed  to  an  interstitial 
sclerosing  inflammation,  with  secondary  nerve  atrophy,  rather 
than  a  primary  nerve  atrophy  with  secondary  interstitial 
change  ;  he  says  "  the  situation  of  the  pathological  process  is 
in  the  optic  nerve,  and  especially  in  that  portion  which  is 
known  as  the  papillo-macular  bundle. 

AMBLYOPIA   FROM   ALCOHOL. 

A  number  of  observers  have  doubted  whether  alcohol  alone 
is  ever  responsible  for  amblyopia,  and  whether  the  cases  which 

1  Ophth.  Review,  1901,  xx.,  151. 

2  Jour,  of  Physiol.,  1900,  xxvi.,  xxxviii. 

3  Ophth.  Review,  1901,  xx.,  189. 

4  v.  Graefe's  Archiv.  f.  Ophth.,  1902,  liii.,  79;  and  1902,  liv.,  68. 

5  Milteilungen    aus   der  Augenklinik.   des    Carolinischen   Medico-Chirurgischen 
Institute  zu  Stockholm,  1906,  viii. 

8  Trans.  Amer.  Ophth.  Soc.,  1897-98,  viii.,  186. 


262  ALCOHOLIC  AM  ELY  OP  I A 

have  been  attributed  to  it  are  not  due  to  other  poisons,  such 
for  example  as  fusel  oil  (Fuchs)  used  as  an  adulterant,  tobacco, 
or  the  results  of  perverted  metabolism  as  in  diabetes. 

Thus,  at  a  special  meeting  of  the  Ophthalmological  Society 
of  the  United  Kingdom  for  the  consideration  of  toxic  ambly- 
opias,1  Nettleship  said  :  "I  have  seen  no  case  of  amblyopia  in 
drinkers  who  did  not  smoke,"  and  with  this  view,  Gunn,  Morton, 
Frost,  Berry  and  Shears  all  concurred. 

A  diagnosis  of  alcoholic  amblyopia  is  certainly  very  seldom 
made  in  England,  and  it  is  of  course  patent,  that  in  an  alcoholic 
subject,  other  causes  of  retro-ocular  neuritis  must  be  excluded 
before  the  condition  can  be  attributed  to  the  alcohol. 

Leber's  disease  has  certainly  been  overlooked  in  some  cases, 
and  unless  the  use  of  tobacco  can  be  entirely  excluded,  a  certain 
degree  of  doubt  as  to  the  true  cause  of  the  visual  defect  must 
remain. 

Nevertheless,  certain  authoritative  observers  in  America  2 
and  Germany  have  been  satisfied  that  amblyopia  may  result 
from  chronic  alcoholism  ;  de  Schweinitz  states  that  "  extensive 
investigations  and  the  reports  of  cases  render  it  certain  that  a 
definite  form  of  amblyopia  is  caused  by  alcohol,  and  is  one  of 
the  conditions  present  in  chronic  alcoholic  poisoning,  even 
when  this  occurs  in  people  who  are  not  addicted  to  the  use  of 
tobacco." 

Peripheral  alcoholic  neuritis  is  often  combined  with  the  loss 
of  sight.3 

Incidence. — In  the  investigation  of  204  cases  of  retro-bulbar 
neuritis,  Uhthoff  4  found  138  were  due  to  toxic  causes,  and  of 
these  138  he  attributed — 

64  to  alcohol. 

45  to  alcohol  and  tobacco. 

23  to  tobacco. 

6  to  other  causes. 
It  has,  however,  to  be  noted,  that  cases  were  attributed 

1  Trans.  Ophth.  Soc.  U.K.,  1887,  vii.,  36. 

2  Connor,  Jour.  Amer.  Med.  Ass.,  1890,  xiv.,  217. 

3  Fuchs,  "  Text-book  of  Ophthalmology,"  1908,  527. 

4  v.  Graefe's  Archiv.f.  Ophth.,  1886,  xxxii.,  iv.,  95;  and  xxxiii.,  i.,  257. 


CLINICAL  SIGNS  263 

to  alcohol  by  him,  when  this  was  taken  to  excess  and  tobacco 
was  used  in  moderation,  and  cases  attributed  to  tobacco 
in  the  contrary  circumstances  ;  as  will  be  seen,  in  forty -five 
cases  Uhthoff  did  not  feel  able  to  discriminate. 

Signs  and  Symptoms. — The  mostfrequent  ophthalmoscopic 
evidence,  according  to  Uhthoff,  is  a  pathological  whiteness  of 
the  temporal  half  of  the  papilla.  Thus,  of  1,000  patients  suffer- 
ing from  severe  alcoholism  in  two  large  asylums  examined  by 
him,  139  had  paleness  of  the  temporal  halves  of  the  disc,  and 
sixty  of  them  suffered  from  amblyopia.  To  control  this 
observation,  Uhthoff  examined  100  normal  men  and  1,000 
lunatics  who  were  not  alcoholic,  and  in  each  case  he  found 
about  1  per  cent,  only  with  paleness  of  the  temporal  part 
of  the  disc.  It  has,  however,  to  be  remembered  that  normally 
the  temporal  half  of  the  disc  is  paler  than  the  nasal  half. 

Occasionally,  haziness  of  the  nerve  head  and  hypersemia  of 
its  surface  are  present,  but  such  findings  as  these  so  readily 
invade  the  broad  field  of  physiological  variation  that  they 
cease  to  be  important. 

In  the  early  stage  there  is  a  relative  central  scotoma  for  red 
and  green  ;  sometimes  complete  or  partial  peripheral  defects 
for  these  colours  occur,  and  sometimes  an  absolute  central 
scotoma  develops.  The  pupil  reactions  are  unaltered.  The 
prognosis  is  said  to  be  less  favourable  than  in  tobacco  and 
other  toxic  amblyopias,  especially  if  there  is  commencing  pallor 
of  the  disc ;  there  is  in  addition  the  great  difficulty  of  insuring 
abstinence  from  drink. 

Histological. — In  a  number  of  instances  the  optic  nerves, 
chiasma,  and  tracts  have  been  carefully  examined  histologically 
by  Samuelsohn,1  Nettleship  and  Edmunds,2  Uhthoff  and  others. 

The  changes  in  alcoholic  cases  are  distinct  from  those  which 
are  seen  in  the  primary  optic  atrophy  of  tabes  or  general 
paralysis.  In  the  latter,  the  connective  tissue  of  the  nerve 
is  not  thickened,  its  network  is  unaltered,  but  the  finest  twigs 
of  the  network  are  atrophic  ;  in  alcoholic  neuritis  these  finest 
twigs  are  thickened  and  proliferated. 

1  v.  Graefe's  Archiv.  f.  Ophth.,  1882,  xxviii.,  i.,  1. 
*  Trans.  Ophth.  Soc.  U.K.,  1881,  i.,  124. 


264  ALCOHOLIC  AM  ELY  OP  I A 

In  alcoholic  cases  the  connective  tissue  network  may  be 
completely  obliterated  and  the  nerve  substance  entirely  dis- 
appear, a  condition  which  does  not  ensue  in  cases  of  primary 
atrophy.  And  again,  in  a  case  of  primary  atrophy  which  affects 
a  part  only  of  the  nerve,  Uhthoff  states  that  normal  fibres  are 
never  present  within  this  part,  whereas  in  alcoholic  cases  they 
are  frequently  seen.  The  preservation  of  these  healthy  fibres 
within  the  diseased  areas  explains  why  alcoholic  neuritis  of  the 
optic  nerve  seldom  leads  to  complete  blindness. 

These  changes  are  thus  summed  up  by  de  Schweinitz  : 

"  Most  of  the  investigations  show  that  the  anatomical  basis 
of  this  affection  consists  of  an  augmentation  of  nuclei,  hyper- 
trophy of  the  connective  tissue,  and  wasting  of  the  nerve  fibres 
of  a  limited  portion  of  the  optic  nerve  known  as  the  papillo- 
macular  bundle  ;  in  fact,  that  there  is  an  interstitial,  sclerosing 
inflammation,  comparable,  according  to  Samuelsohn,  to  the 
same  pathological  process  which  alcohol  produces  in  the  liver 
in  interstitial  hepatitis." 

The  position  of  the  papillo-macular  bundle  of  fibres  is  as 
follows,  quoted  from  de  Schweinitz  : — 

"  The  papillo-macular  bundle,  according  to  Bunge's  nomen- 
clature, which  consists  of  those  fibres  in  the  optic  nerve 
which  supply  the  retina  between  the  macula  lutea  and  the 
papilla,  and  are  more  or  less  diseased  in  all  of  these  cases, 
lie  in  the  temporal  portion  of  the  nerve  tip  in  a  wedge-shaped 
segment. 

"  The  triangular  portion  is  directed  with  its  apex  towards 
the  vessels  and  occupies  about  one-third  of  the  surface  of  the 
papilla. 

"As  it  pursues  its  way  through  the  orbital  portion,  it 
gradually  approaches  the  axis  of  the  nerve  which  it  reaches  in 
the  optical  canal. 

"  At  the  front  of  the  chiasm  it  occupies  its  upper  and  inner 
portion,  but  in  the  tracts  it  sinks  to  the  central  portion,  and 
remains  there  till  it  arrives  at  the  brain. 

"  With  the  entrance  of  the  vessels  into  the  optic  nerve  trunk, 
the  cross-section  of  the  diseased  fibres  quickly,  according  to 
Samuelsohn,  or  gradually  according  to  other  observers,  pre- 


WOOD  ALCOHOL  265 

sents  the  form  of  an  oval,  reaching  from  the  temporal  side  of 
the  optic  nerve  more  towards  the  centre. 

"  In  the  intra-cranial  portion  of  the  optic  nerve  the  bundle 
first  reaches  a  central  position,  whilst  in  the  chiasm  and  the 
tractus  it  occupies  the  positions  just  noted." 

Bunge  believes  that  the  papillo-macular  bundle  divides  into 
two  portions  in  the  posterior  portion  of  the  chiasm,  an  upper 
and  a  lower  part,  while  Vossius  finds  this  division  in  the  tractus. 

WOOD   ALCOHOL   BLINDNESS. 

Very  rapid,  severe,  and  permanent  damage  to  the  sight  may 
occur  from  the  absorption  of  wood  alcohol  into  the  system 
either  when  taken  by  the  mouth  or  when  absorbed  by 
inhalation.  It  is  a  form  of  poisoning  which  seems  to  be  of 
C  more  frequent  occurrence  in  America  and  Germany  than  in 
EnglandJ 

Blindness  follows  rapidly  on  the  absorption  of  the  drug,  the 
pupil  is  dilated,  there  is  intense  oedema  of  the  retina,  and 
neuritis  l  followed  by  optic  atrophy. 

In  some  of  the  less  severe  cases,  after  blindness  has  persisted 
for  a  few  days,  recovery  sets  in  and  is  occasionally  complete. 
Ziegler  reports  a  case  in  which  recovery  commenced  after  so 
long  as  two  months.  In  more  severe  cases  death  is  not  an 
uncommon  sequel  ;  in  others,  the  blindness  persists  and 
becomes  absolute  and  permanent,  the  optic  nerve  undergoing 
atrophy. 

In  some  cases  the  alcohol  has  been  taken  by  mistake,  in 
some  it  has  been  used  as  an  adulterant,  and  in  some  it  has 
been  absorbed  by  inhalation  during  some  commercial  process. 
Wilmer  2  reports  the  case  of  a  soldier  who  took  4  drachms 
in  mistake  for  whiskey ;  next  morning  his  sight  was  poor, 
and  on  the  fourth  day  he  was  practically  blind,  and  so 
remained.  Gruening  3  says  :  "  Wood  alcohol  poisoning  is  very 
common  throughout  the  States  ;  wood  alcohol  is  used  in  many 

1  Fridenberg,  Trans.  Amer.  Ophth.  Soc.,  1909-11,  xii.,  514. 

2  Trans.  Amer.  Ophth.  Soc.,  1909-11,  xii.,  523. 

3  Trans.  Amer.  Ophth.  Soc.,  1909-11,  xii.,  522. 


266  QUININE  AM  AU  EOS  IS 

beverages."  Mendel 1  reports  the  accidental  poisoning  of  a  large 
gathering  of  individuals  ;  130  were  transferred  to  hospital, 
of  whom  fifty-one  died ;  many  became  blind  before  death . 
Four  patients  were  exhibited,  all  of  whom  had  pale,  white 
atrophic  discs. 

As  instances  of  poisoning  by  inhalation,  Tyson  2  reports  the 
case  of  three  men  who  were  occupied  on  shellacing  the  interior 
of  a  large  vat,  the  shellac  being  dissolved  in  wood  alcohol  ;  two 
of  them  died  and  one  became  blind :  de  Schweinitz  3  reports 
the  case  of  a  man  who  worked  in  a  close  space  with  Columbian 
spirits  which  contains  about  95  per  cent,  of  wood  alcohol  ;  he 
also  became  rapidly  and  permanently  blind  from  inhaling  the 
spirit. 

Birch -Hirschf eld 4  experimenting  on  four  dogs  and  three 
monkeys  found  marked  changes  in  the  retinal  ganglia  before 
any  morbid  change  could  be  traced  in  the  optic  nerve.  He 
believes  that  the  primary  ^action  is  upon  the  ganglion  cells; 
and  that  an  ascending  degeneration  of  the  nerve  follows,  the 
changes  in  the  neuroglia  and  septa  being  secondary  only. 

Fridenberg  explains  the  action  of  the  alcohol  as  due  "to  an 
intense  oedema  with  some  neuritis,  followed  rapidly  by  optic 
nerve  atrophy,  and  due  to  formalin  or  formic  acid  circulating 
in  the  blood,  as  a  result  of  incomplete  combustion  of  methyl 
alcohol  ingested."  Kelly  5  reports  the  case  of  one  of  a  coterie 
of  six  congenial  spirits  who  were  taken  ill  after  a  convivial 
evening  together.  His  patient  was  suffering  from  severe  loss  of 
sight  after  taking  two  Bronx  cocktails,  and  of  the  five  remaining, 
four  were  dead  and  one  was  blind. 

QUININE. 

The  susceptibility  of  different  individuals  towards  the  toxic 
effects  of  quinine  varies  greatly.  I  have  seen  a  woman  who, 
not  knowing  of  this  effect  of  the  drug,  stated  that  if  she  took  a 

1  Berlin  Ophth.  Gesells.,  1912. 

*  Trans.  Amer.  Ophth.  Soc.,  1912-14,  xiii.,  146. 

3  Trans.  Amer.  Ophth.  Soc.,  1909-11,  xii.,  523. 

«  v.  Graefe's  Archiv.  f.  Ophth.,  1902,  liv.,  68. 

5  Quoted  Clairborne,  New  York  Acad.  of  Med.,  Ophth.  Section,  1919. 


CLINICAL  SIGNS  267 

^r 
r 

teaspoonful  of  the  ammoniated  tincture,  i.e.,  l"  grain  of  the 
drug,  her  sight  has  dimmed  for  a  quarter  of  an  hour  ;  on  the 
other  hand,  what  large  doses  are  tolerated  by  many  malarial 
patients  !  and  it  is  stated  that  so  much  as  an  ounce  has  been 
taken  without  disastrous  results. 

This  drug  is  taken  by  some  women  as  an  abortifacient,  a 
practice  which  supplies  a  proportion  of  the  ophthalmic  cases 
that  one  sees.  On  asking  a  woman  why  she  had  taken  it,  she 
said  :  "  Oh  !  you  know  !  "  She  had  evolved  a  formula  for  its  use  ; 
if  she  missed  a  period  she  took  a  penn'orth,  and  if  she  did  not 
abort  within  three  days  she  took  three -penn'orth  of  the  drug, 
a  quantity  which  no  doubt  varied  greatly  with  the  generosity 
of  the  druggist. 

\  asked  her  if  many  women  took  it.  She  said  :  '  Yes,  I 
know  a  woman  who  is  experimenting  with  it  now." 

The  late  George  Coats  had  a  man  under  observation  for  some 
years  who  went  blind  from  having  taken  within  twenty-four 
hours,  two  bottles  of  a  proprietary  orange  and  quinine  wine.  I 
saw  him  four  years  afterwards ;  his  fields  were  greatly 
restricted,  the  retinal  vessels  were  very  attenuated,  and  the 
discs  were  white. 

Bruns  reports  the  case  of  a  child  of  three  who  was  given  five 
grains  by  the  rectum  within  eighteen  hours  for  remittent  fever, 
and  in  whom  ophthalmoscopic  changes  and  visual  defects  per- 
sisted ten  years  later. 

Harlan  *  reports  the  case  of  a  man  of  thirty -four  who  took 
200  grains  of  quinine  and  who  was  blind  for  ten  days  ;  improve- 
ment then  set  in,  and  seven  weeks  later  the  visual  fields  extended 
outwards  to  about  25  degrees  all  round,  and  his  central  vision 
was  largely  restored. 

Symptoms. — The  first  toxic  symptom  is  tinnitus  and  deaf- 
ness, which  most  often  is  temporary  only,  loss  of  sight  follows 
within  a  few^hours  and  is  often  severe  and  may  be  absolute  ; 
the  pupils  are  widely  dilated  and  are  inactive  to  light ;  the 
retinal  vessels  show  extreme  tenuity,  and  the  disc  and  fundi  are 
very  pale  ;  complete  loss  of  consciousness  may  occur. 

The  degree  to  which  sight  is  affected  varies  greatly,  from  a 

1  Ophth.  Record,  1897,  vi.,'113. 


268  QUININE  AMAUROSIS 

temporary  obscuration  to  complete  blindness,  but  in  all  cases 
the  tendency  is  towards  recovery,  and  it  is  doubtful  whether 
absolute  permanent  blindness  ever  ensues  in  man. 

Onset. — The  onset  may  be  gradual  and  take  several  days  for 
its  development,  but  it  is  more  usual  for  it  to  occur  within  a 
few  hours  ;  on  the  other  hand,  with  large  doses  it  is  often  quite 
sudden. 

i  A  sea  captain  feeling  "  a  cold  "  coming  on,  knocked  out  some 
quinine  on  to  the  palm  of  his  hand  and  swallowed  it.  Seven 
hours  later,  whilst  looking  out  to  sea,  it  was,  he  said,  exactly 
as  if  a  shutter  had  been  let  down  over  his  right  eye,  and  one 
second  later  the  sight  of  the  left  eye  was  blotted  out  as  suddenly  ; 
it  was  twenty-four  hours  before  he  saw  even  the  glimmer  of 
an  electric  light. 

Its  duration  may  be  a  few  hours  or  even  less,  but  in  cases  of 
any  severity,  there  is  a  period  of  total  blindness  in  which  light 
perception  is  absent,  and  this  period  may  be  from  a  day  or  two 
to  several  weeks.  In  a  case  of  Dewey's  l  this  blindness  lasted 
for  three  months,  and  at  the  end  of  eighteen  months  there  were 
still  serious  visual  defects. 

In  dogs  experimented  on  by  de  Schweinitz  there  was  in  some 
cases  still  total  blindness  at  the  end  of  two  months. 

The  visual  fields  show  a  marked  peripheral  constriction,  and 
except  in  the  mildest  cases  some  degree  of  this  defect  is  per- 
.-/  manent ;  central~vision  is  affected  much  less  severely. 
/  j      Barabaschew  2  experimented  on  six  of  his  willing  colleagues 
'   /    in  order  to  observe  the  effects  upon  healthy  individuals.     Each 
took  from  40  to  60  grains  of  quinine  hydrochlorate,  and  three 
of  them  presented  definite  symptoms .     The  phenomena  observed 
were  : — 

Pallor  of  the  face  and  conjunctiva. 

Marked  contraction  of  the  retinal  vessels  and  pallor  of 

the  optic  discs. 
Diminution  of  visual  acuity. 
Concentric  constriction  of  the  visual  fields. 

1  Trans.  Med.  Assoc.  Missouri,  1882,  161. 

2  Westnik  Ophth.,  1891,  viii.,  i.,  14;  and  Archiv.f.  Augenheilk.,  1891,  xxiii.,  91. 


EXPERIMENTAL    WORK  269 

And  of  less  recognised  symptoms  : — 

Temporary  increase  of  visual  acuity  lasting  some  hours. 
Contraction  of  the  pupil  lasting  some  time,  followed  by 
moderate  dilatation. 

Complete  amaurosis  occurred  in  one  case  only,  and  lasted  a 
very  short  time.  Marked  ischaemia  of  the  retina  occurred  in 
all  cases,  and  in  two  the  pallor  resembled  optic  atrophy. 

In  those  cases  in  which  symptoms  of  any  marked  degree 
persist,  the  special  ophthalmoscopic  feature,  in  addition  to 
pallor  of  the  disc,  is  a  great  constriction  of  the  retinal  arteries. 

Several  observers  have  commented  upon  the  liability  to 
relapse  after  small  doses  of  the  drug. 

In  some  cases  in  the  early  stage,  according  to  Gruening,1  a 
cherry  red  spot  at  the  maculae  may  be  seen  with  surrounding 
grey  retinal  haze ;  this,  however,  was  not  observed  by 
Barabaschew. 

Experimental. — Experimental  work  on  dogs  by  Holden 2 
and  de  Schweinitz  3  shows  that  as  early  as  the  third  day  there 
is  marked  degeneration  in  the  ganglion  cells  of  the  retina,  and 
later,  by  the  forty -second  day,  only  a  few  normal  ganglion  cells 
remained  ;  consequent  upon  this,  degeneration  takes  place  in 
the  optic  nerve  fibres  extending  to  the  mid-brain  ;  the  other 
layers  of  the  retina  are  not  affected. 

In  the  case  of  a  dog,  blind  for  sixty-one  days,  de  Schweinitz 
says  :  "In  none  of  the  sections  is  there  any  indication  of 
disease  of  the  vessels  of  the  uveal  tract."  He  looks  upon  the 
process  as  due  to  the  direct  action  of  the  drug  upon  the 
peripheral  vessels,  which,  by  deprivation  of  blood,  causes  the 
degeneration  of  the  nerve  cells.  He  shows  that  there  are  no 
changes  in  the  cells  of  the  visual  cortex.  Organic  changes  in 
the  vessels  is  a  late  and  secondary  process. 

De  Schweinitz  shows  sections  of  the  ciliary  ganglion  and  the 
third  nerve  which  are  entirely  free  of  change,  thus  emphasising 
how  selective  is  the  action  of  the  drug  upon  the  retina. 

1  Quoted  by  de  Schweinitz,  "Toxic  Amblyopias,"  Philadelphia,  1896,  184. 

2  Trans.  Amer.  Ophth.  Soc.,  1897-98,  viii.,  405. 

3  Trans.  Amer.  Ophth.  Soc.,  1891-93,  vi.,  23  ;  1906-8,  xi.,  338  ;   and  Trans.  Coll. 
Phys.  of  Philadelphia,  1890,  xii.,  185. 


270  AM  ELY  OP  I A   DUE   TO  LEAD 

SODIUM   SALICYLATE. 

Temporary  loss  of  sight  occurs  at  times  from  large  doses  of 
the  salicylates ;  the  effects  are  similar  to  those  of  quinine,  but 
are  less  severe  in  degree  and  probably  are  never  permanent. 

The  arteries  are  reduced  in  size,  the  pupils  are  dilated  and 
inactive,  and  deafness  and  tinnitus  occur.  Recovery  is  more 
rapid  and  complete  than  in  quinine  blindness. 

De  Schweinitz  l  experimented  on  dogs  and  showed  that 
blindness  could  be  produced  in  them.  He  says  :  "  It  is  possible 
to  produce  partial  blindness  with  large  doses  of  salicylic  acid  or 
salicylate  of  soda,  the  ophthalmoscopic  appearances  resembling 
in  minor  degree  those  seen  in  quinine  amaurosis." 

Gatti 2  reports  the  case  of  a  sixteen-year-old  girl  who  was  given 
8  grammes  of  salicylate  of  soda  in  ten  one -hourly  doses.  After 
receiving  the  last  dose  she  fell  asleep,  and  awoke  blind ; 
there  were  no  marked  ophthalmoscopic  signs  ;  in  twenty- 
four  hours  vision  was  restored.  Snell  3  reports  the  case  of  a 
girl  of  nine  who  was  given  150  grains  of  the  sodium  salt  in 
sixty  hours  for  acute  rheumatism  ;  she  became  quite  blind  and 
was  unable  to  tell  light  from  dark,  and  this  condition  remained 
till  her  death.  The  pupils  reacted  to  light,  the  fundi  were 
normal,  there  was  no  deafness  or  tinnitus, 

LEAD. 

It  has  for  long  been  known  that  the  vision  and  ocular  muscles 
may  be  seriously  affected  in  poisoning  by  lead.  Sometimes  the 
ocular  complications,  especially  retinitis  and  optic  neuritis, 
arise  as  a  manifestation  of  vascular  or  renal  disease  which 
is  produced  by  the  poison,  and  Jeaffreson 4  haa  expressed 
doubt  whether  such  lesions  can  be  produced  by  the  lead  direct ; 
that,  however,  such  may  occur  seems  to  have  been  made 
clear  by  Oliver.5 

Lead  may  be  introduced  into  the  body  by  several  paths  ;  it 
may  be  absorbed  through  the  skin,  or  inhaled  as  dust,  or  taken 

"  Toxic  Amblyopias,"  Philadelphia,  1896,  205. 

Quoted  by  de  Schweinitz. 

Trans.  Ophth.  Soc.  U.K.,  1901,  xxi.,  306. 

Brit.  Med.  Jour.,  1886,  i.,  390. 

Brit.  Med.  Jour.,  1885,  ii.,  731. 


CLASSIFICATION  OF  CASES  271 

into  the  system  by  the  mouth  ;  most  cases  occur  as  a  result  of 
some  industrial  process  in  which  lead  is  used,  and  amongst 
individuals  who  are  regardless  of  the  precautions  advised,  or 
are  careless  as  to  their  observance. 

Gibson  *  reports  an  epidemic-like  occurrence  in  children,  of 
whom  sixty-two  were  admitted  to  the  Brisbane  Hospital  for 
Sick  Children  in  three  years.  In  them  the  source  of  the  lead  was 
traced  to  the  lead  carbonate  in  the  white  paint  with  which  the 
railings  around  the  houses  were  painted. 

Frequency. — Amongst  Gibson's  sixty-two  cases,  thirteen  had 
eye  symptoms.  Amongst  138  cases  of  toxic  amblyopia, 
Uhthoff  found  one  only  due  to  lead,  and  Norris  2  found  three 
cases  of  saturnine  amblyopia  amongst  15,000  cases  of  eye 
diseases. 

Males,  in  general,  are  more  liable  to  suffer  than  are  females 
in  the  proportion  of  forty-six  to  twelve,3  owing  to  the  occupa- 
tional incidence  of  the  poisoning  ;  patients  of  all  ages  may  be 
affected. 

There  is  great  variation  in  the  time  interval  between  the  first 
exposure  to  the  poison  and  the  onset  of  eye  symptoms,  varying, 
no  doubt,  with  the  thoroughness  of  the  precautionary  measures, 
but  also  depending  upon  the  varying  susceptibility  which 
different  individuals  possess  ;  tfyus,  it  is  stated  by  de  Schweinitz 
that  symptoms  may  arise  within  twelve  days  of  exposure  ;  on 
the  other  hand,  there  may  be  immunity  at  the  end  of  many 
years. 

It  is  quite  unusual  to  find  eye  symptoms  without  other 
undoubted  evidence  of  chronic  plumbism  being  present, 
e.g.,  wrist  drop,  colic,  headaches,  blue  line  in  the  gum,  lead  in 
the  urine,  etc. 

The  most  useful  classification  of  the  types  of  ocular  lesion  is 
that  given  by  de  Schweinitz,  thus  :— 

(1)  Transient  amblyopia  without  ophthalmoscopic  change, 
due  to  an  anaesthetic  effect  of  the  lead  on  the  retina  and  optic 
nerve,  and  not  unlike  amaurosis  from  uraemia  without  fundus 

lesions. 

1  Brit.  Med.  Jour.,  1908,  ii.,  1488. 

2  Quoted  Brose,  Archives  of  Ophth.,  1915,  xliv.,  27. 

3  Stood,  v.  Graefe's  Archiv.,  1884,  xxx.,  iii.,  215. 


272  AMBLYOPIA   DUE   TO  LEAD 

This  type  is  clearly  defined,  the  onset  is  rapid,  and  the 
recovery  rapid — within  a  few  days — and  complete  ;  it  is 
referred  to  by  Loewe  l  and  Galezowski.2 

(2)  Permanent  amblyopia  without  distinct  fundus  changes, 
or  at  most,  some  hyperaemia  of  the  nerve-tip  and  undue  filling 
of  the  retinal  circulation,  due  to  a  retro-bulbar  neuritis  analo- 
gous  to  that   occurring   under  the   influence   of    other  toxic 
agents.     This   type   may   terminate   in   blindness   from   optic 
nerve  atrophy. 

(3)  Optic  neuritis,  or  neuro-retinitis,  either  specifically  due 
to  the  lead,  or  secondary  to  changes  in  the  brain  or  kidneys. 

-  (4)  Optic  nerve  atrophy,  either  consecutive  to  a  plumbic 
papillitis,  or  due  to  the  primary  effect  of  the  lead  on  the  visual 
apparatus. 

(5)  Various  types  of  retinitis,  often  due  to  lead  nephritis,  but 
also  primary,  and  appearing  in  the  form  of  vasculitis  and 
perivasculitis,  as  described  by  Oliver.3 

Of  thirty-four  cases  examined  by  Stood,4  50  per  cent, 
suffered  from  optic  neuritis  or  optic  atrophy,  and  of  sixty -four 
cases  analysed  by  de  Schweinitz,  thirteen  had  optic  neuritis, 
four  neuro-retinitis,  seventeen  optic  atrophy,  and  seventeen 
were  stated  to  be  blind  without  a  description  of  the  ophthal- 
moscopic  appearances  ;  in  nineteen  cases  the  fundi  were  normal, 
and  therefore  belonged  to  either  class  1  or  2. 

Subjective" colour  sensations  are  not  infrequent,5  and  various 
forms  and  degrees  of  colour  Tmndness  are  seen.  Debility  of 
accommodative  power  is  common,  and  nystagmus  is  sometimes 
present.  The  visual  fields  may  be  concentrically  constricted, 
peripheral  scotomata  may  occur,  and  typical  central  scotomata, 
such  as  are  found  in  intoxication  amblyopia,  are  described  by 
de  Schweinitz.  The  external  ocular  muscles  are  not  infrequently 
paralysed,  and  of  them  the  external  rectus  is  the  most  common. 

Prognosis. — This  will  obviously  depend  upon  the  stage  to 
which  the  disease  has  progressed  when  the  patient  comes  under 

1  Archives  of  Ophth.,  1906,  xxxv.,  164. 

2  Archives  generates  de  me'dicin,  1906,  i.,  1613. 

8  "  Lead  Poisoning  in  its  Acute  and  Chronic  Forms,"  Edinburgh  and  London. 
1891,  76. 

*  v.  Graefe's  Archiv.  f.  Ophth.,  1884,  xxx.,  iii.,  215. 
'  Folker,  Trans.  Ophth.  Soc.  U.K.,  1899,  xix.,  257. 


NITRATE  OF  SILVER  273 

observation,  and  will  be  guidecrty  the  presence  or  absence  of 
ophthalmoscopic  evidence  of  organic  change  in  the  nerve. 

Little  improvement  can  be  expected  when  the  optic  disc  is 
already  white  and  atrophic  ;  where  neuritis  or  neuro-retinitis 
is  present,  removal  from  the  poison  holds  out  a  good  prospect 
of  improvement  ;  and  in  the-  cases  of  complete  blindness  of 
sudden  onset,  complete  recovery  may  be  predicted. 

Of  five  cases  reported  by  Folker,  four  went  blind  completely 
and  permanently  with  atrophic  discs  and  greatly^  attenuated 
vessels  ;  Hutchinson  x  reports  five  cases  of  optic  neuritis  with 
secondary  atrophy. 

NITRATE   OF   SILVER. 

There  are  no  authentic  instances  of  silver  nitrate  producing 
a  toxic  amblyopia,  but  Gowers  2  states  that  its  effect  is  closely 
analogous  to  that  of  lead,  and  therefore  it  isC  highly  probable 
that  the  same  ocular  changes  may  result  in  some  cases. 

It  is  well  known  that  staining  of  the  conjunctiva  may  result 
from  the  prolonged  use  of  silver  nitrate  or  the  organic  com- 
pounds, protargol  and  argyrol.  I  have  seei^'argyrosis  of  the 
conjunctiva  which  had  developed  in  three  months,  as  a  result 
of  the  use,  three1  times  weekly,  of  a  I  per  cent,  solution  of 
argyro/  I  have  recently  seen  a  man  who  was  occupied  in  the 
manufacture  of  silver  nitrate,  the  whole  of  the  exposed  parts  of 
whose  body,  face  and  hands,  were  as  dark  as  a  negro's  but  of  a 
duller  brown.  His  conjunctiva  was  of  a  similar  colour,  and 
not  only  so,  but  the  whole  of  the  surface  of  the  cornea  was 
stained  a  characteristic  dull  brown  hue.  He  stated  that  the 
colour  was  distinctly  fainter  after  a  holiday,  and  that  he  had 
discarded  protective  goggles  because  of  the  grotesque  effect 
produced,  the  protected  parts  soon  becoming  pale,  whilst  the 
rest  of  his  face  was  black.  He  was  aged  seventy-three  and 
was  robust  ;  his  two  predecessors  died  at  the  ages  of  seventy- 
two  and  seventy-seven,  one  of  bronchitis  and  one  of  "  com- 
plications "  ;  in  neither  of  them,  so  far  as  he  knew,  did  either 
epitheliomata  or  warts  develop  in  the  skin. 

1  Royal  London  Ophth.  Hosp.  Reports,  1873,  vii.,6. 

2  "  Medic.  Ophthal.,"  1904,  266. 

vn  18 


274  IODOFORM 

IODOFORM. 

A  few  cases  of  marked  loss  of  sight  are  reported  as  a  result 
of  the  action  of  iodoform.  In  some  cases  the  drug  has  been 
taken  by  the  mouth,1  in  other  cases  it  has  been  absorbed  as 
a  result  of  external  application.2 

In  Priestley  Smith's  case  —  a  man  of  thirty-one  with  tuber- 
culous pleurisy  and  peritonitis-^1,000  grains  were  administered 
by  the  mouth  in  forty-one  days  ;  during  the  last  few  days  he 
was  taking  thirty  -two  grains  a  day);  he  then  developed  ambly- 
opia  and  other  signs  of  iodoform  poisoning.  In  Critchett's2 
case  the  drug  was  being  used  as  a  dressing  for  carcinoma  of 
the  breast  ;  in  Mohr's  2  cases  tuberculous  "abscesses  were  injected 
with  iodoform  emulsion. 

The  symptoms  of  the  condition  are  loss  of  central  vision 
with  no  constriction  of  the  visual  fields,  and  no  constant  or 
conspicuous  ophthalmoscopic  'changes  ;  there  is  a  general 
similarity  between  these  symptoms  and  those  of  tobacco 


The  prognosis  seems  to  be  good  in  all  cases  when  the  drug 
is  stopped,  the  time  required  for  recovery  varying  with  the 
amount  taken  and  the  duration  of  the  symptoms.  Priestley 
Smith's  case  was  very  nearly  normal  in  three  months  ;  in 
another  case  quoted  by  de  Schweinitz  recovery  occurred  in 
eight  days. 

BISULPHIDE   OF   CARBON. 

Chloride  of  sulphur  dissolved  in  bisulphide  of  carbon  is 
largely  used  in  Parke's  vulcanising  process,  and  in  certain  other 
processes  of  manufacture,  but  it  is  only  in  the  former  process 
that  toxic  symptoms  arise,  since  in  the  latter  closed  vessels  are 
used. 

This  chemical  was  used  for  a  short  time  by  Simpson  as  a 
general^ihsesthetic  . 

1  Priestley  Smith,  Ophth.  Review,  1893,  xii.,  101  ;   Hutchinson,  New  York  Med. 
Jour.,  1886,  xliii.,  16. 

2  yLohT,Archiv.f.Augenheilk.,  1902,  xlv.,  184  ;  Critchett,  Trans.  Ophth.  Soc.  U.K., 
1898,  xviii.,383;  Valude,  Annal.  d'Oculist.,  1893,  cix.,  378. 


BISULPHIDE  OF  CARBON  275 

The  first  investigations  into  the  profound  toxaemia  produced 
were  carried  out  by  Delpech,1  who  reported  thirty-three  cases, 
fifteen  of  whom  suffered  from  amblyopia.  Cases  were  then 
reported  by  a  number  of  observers,  and  in  1885  a  special 
committee  of  the  Ophthalmological  Society  of  the  United 
Kingdom  was  appointed  to  investigate  thoroughly  the  subject. 
They  tabulated  twenty -four  cases  collected  from  the  literature. 
That  the  path  of  entrance  of  the  poison  is  through  the  lungs  was 
clearly  established,  for  men  who  inhale  the  vapour  are  affected, 
though  the  liquid  never  comes  into  contact  with  their  skin,  whilst 
those  who  have  their  hands  immersed  in  the  fluid  do  not 
develop  symptoms  if  proper  masks  are  worn. 

Symptoms. — These  may  be  divided  into  three  stages,  as 
described  by  the  committee. 

The  earliest  is  a  heavy  pain  in  the  forehead  and  temples 
which  may  precede  other  symptoms  by  months  or  years. 
This  is  succeeded  by  a  stage  of  well-marked  exaltation  of 
spirits ;  vision  is  sometimes  affected  during  this  stage,  the 
trouble  often  being  temporary  and  coming  on  towards  the  end 
of  the  day ;  hearing  is  affected  more  often. 

The  third  stage  is  characterised  by  depression,  loss  of  appe- 
tite and  insomnia  ;  visual  troubles  become  more  frequent,  a 
fog  comes  before  the  eyes  which  is  most  marked  in  broad  day- 
light. The  pupil  reactions  remain.  There  appears  to  be  no 
constant  ophthalmoscopic  change  ;  pallor  of  the  discs,  slight 
neuritis,  partial  or  complete  atrophy,  and  normal  discs  have 
all  been  described.  There  is  great  lowering  of  central  visual 
acuity,  the  scotoma  being  most  marked  for  red  and  green  ;  the 
peripheral  fields  are  complete.  The  visual  affection  never  occurs 
without  well-marked  general  toxic  symptoms. 

When  the  sufferers  are  removed  from  the  fumes,  they  recover 
slowly,  but  a  relapse  is  certain  if  they  resume  the  same  work. 

Of  the  cases  investigated  by  the  special  committee,  33  per 
cent,  recovered,  25  per  cent,  improved,  and  20  per  cent,  showed 
little  or  no  improvement  ;  very  similar  figures  were  obtained 
by  de  Schweinitz. 

1  "  Memoire  sur  les  accidents  que  developpe  dans  les  ouvriers  en  Caoutchouc 
1'inhalation  du  Sulfure  de  Carbone  en  Vapeur,"  Paris,  1856  and  1863. 

18—2 


276  NITRO-BENZOL 

NITRO-BENZOL. 

i 
This  chemical  is  used  in  the  manufacture  of  certain  explosives, 

and  during  the  process  fumes  ^are  given  off  which  in  a  few  cases 
give  rise  to  toxic  symptoms,  amongst  which  are  affections  of 
sight.  Cases  are  reported  by  White,1  Snell,2  and  others. 

In  addition  to  the  inhalation  of  fumes,  the  chemical  may 
enter  the  lungs  as  a  fine  dust,  or  may  be  absorbed  through  the 
skin. 

The  general  ^symptoms  of  nitrobenzol  poisoning  include 
headache,  muscular  weakness,  cyanosis,  dilated  pupils,  rapid 
shallow  respirations,  and  a  thready  failing  pulse.  The  ocular 
symptoms  are  :  diminished  central  acuity,  contraction  of  the 
visual  fields,  and  sometimes  a  colour  scotoma.  The  ophthal- 
moscopic  signs  are  a  darker  "colour  of  the  fundus  than  normal, 
venous  hypersemia,  the  veins  being  dark,  full  and  tortuous,  and 
pallor  of  the  discs  with  slight  blurring  of  their  margins. 

It  is  stated  by  Snell  that  the  dark  colour  of  the  fundus  occurs 
in  those  who  work  in  the  factories,  even  though  their  sight  is 
not  affected. 

The  prognosis  is  good  if  the  patient  is  removed  from  access 
to  the  poison. 

ANILINE. 

The  symptoms  which  arise  from  this  compound,  both  ocular 
and  general,  are  much  similar  to  those  which  are  caused  by 
nitre-benzol.  In  addition,  intense  pigmentation  of  the  cornea 
and  conjunctiva  may  occur  amongst  workers  in  aniline  dyes.3 

CARBOLIC   ACID. 

A  case   of   total   blindness  followed   by  complete   recovery 

is    reported    by    Nieden 4    in  a    man    in    whom    the    cavity 

of  an  empyema  was  washed  out  with  a  S'^per  cent,  solution 
of  phenol. 

1  Practitioner,  1889.  xliii.,  14:  and  Provincial  Mcd.  Jour.,  1802,  xi.,  462. 

2  Brit.  Med.  Jour.,  1894,  i.,  449. 

3  Mackinlay,  Trans.  Ophtk.  Soc.  U.K.,  1880,  vi.,  144. 

4  P,<  din  kiln.  Wochenschr.,  1882,  xix.,  748. 


NAPHTHALIN  277 

OCHRONOSIS. 

The  term  "  ochronosis  "  is  applied  to  a  burnt  sienna-like  colora- 
tion of  the  cartilaginous,  elastic,  and  connective  tissues  of  the 
body,  and  in  some  cases  the  triangular-shaped  portion  of  the 
sclerotic  at  each  side  of  the  cornea  as  it  appears  in  the  palpebral 
fissure  is  involved  in  this  coloration. 

Albrecht  showed  that  it  occurred  in  the  subjects  of  alkapto- 
nuria  ;  both  it  and  the  urinary  abnormality  may  result  from  the 
prolonged  external  application  of  carbolic  dressings  to  wounds. 

In  alkaptonuria  the  urine  is  normal  in  colour  as  it  is  passed, 
but  it  slowly  acquires  a  deep  brown  colour,  and  ultimately 
becomes  almost  black.  It  is  darkened  by  the  addition  of 
alkalies,  and  reduces  Fehling's  solution.  Sir  Archibald  Garrod 
states  that  alkaptonuria  is  more  common  in  males  than  in 
females,  and  that  it  dates  from  birth  or  early  childhood.  Its 
occurrence  is  not  of  any  serious  import. 

NAPHTHALIN. 

It  was  found  by  Bouchard,  that  if  large  doses  of  naphthalin 
were  administered  to  rabbits,  double  cataract  developed  in  the 
course  of  a  week  or  two. 

I  have  examined  the  eyes  of  several  patients  who  under  the 
care  of  Dr.  J.  H.  Drysdale  had  been  treated  for  leukaemia  over 
long  periods  with  considerable  doses  of  naphthalin  tetrachloride  ; 
in  no  case  had  cataract  developed.  A  similar  negative  result 
was  found  by  Burton  in  typhoid  fever  patients  treated  at  the 
Alexian  Hospital,  Chicago,  with  the  same  drug  for  several  weeks. 

Peters 1  states  that  an  increase  of  salts  in  the  aqueous  humour 
results  from  the  administration  of  naphthalin,  and  suggests  that 
the  disturbance  of  osmosis  between  the  lens  and  aqueous  so  pro- 
duced is  responsible  for  the  development  of  lenticular  opacities. 

ANTIPYRIN. 

This  drug  has  caused  temporary  blindness  in  a  few  cases. 
Hotz 2  mentions  four  such  ;  two  patients  went  blind  after 

1  Trans.  Heidelberg  Ophth.  Soc.,  1902,  20. 

2  Archiv.  of  Ophth.,  1906,  xxxv.,  160. 


278  SANTONIN 

taking  15  grains  of  the  drug,  one  within  five  minutes,  and  one 
in  twenty  minutes  ;  both  rapidly  recovered.  Another  lost  his 
sight  in  forty-eight  hours  after  taking  130  grains  ;  the  discs  were 
pale  and  the  vessels  narrow  ;  complete  recovery  occurred. 

De  Schweinitz  describes  visual  phenomena  similar  to  those 
which  occur  as  prodromata  of  migraine  ;  these  effects  he  pro- 
duced on  himself  by  taking  large  doses  of  the  drug. 

SANTONIN. 

Within  a  quarter  of  an  hour  of  taking  even  non-toxic  doses 
of  this  drug,  everything  within  the  field  of  vision  is  tinged  a 
bright  yellow,  as  if  it  were  seen  through  a  yellow  glass. 

Knies,1  from  experiments  upon  himself,  found  that  a  short 
period  of  violet  vision  preceded  the  yellow  vision.  He  describes 
the  colour  as  citron  yellow ;  occasionally  green  takes  the  place 
of  the  yellow  colour.  Rose  2  has  made  most  elaborate  in- 
vestigations into  this  form  of  chromatopsia.  According  to 
Franceschi,3  coloured  vision  occurs  only  if  the  drug  is  taken 
before  being  exposed  to  light  ;  if  it  is  dissolved  and  exposed  to 
light  for  twenty -four  hours,  the  solution  becomes  yellow  and  does 
not  then  produce  xanthopsia,  but  this  is  not  in  accord  with  com- 
mon experience.  Except  where  severe  symptoms  of  santonin 
poisoning  arise,  there  are  no  objective  or  permanent  ocular 
effects. 

ASPIDIUM    (FILIX   MAS). 

This  drug  is  usually  given  either  in  the  form  of  a  liquid 
extract  or  an  oleoresin,  as  an  anthelmintic.  It  is  a  violent 
gastro-intestinal  poison  and  has  caused  death  when  given  in 
excessive  doses  ;  short  of  this,  however,  it  has  been  responsible 
for  a  number  of  cases  of  loss  of  sight,  3  grammes  of  the 
extract  given  daily  for  twelve  consecutive  days  having  caused 
blindness  (de  Schweinitz). 

1  Archiv.f.  Augenheilk.,  1888,  xviii.,  64. 

2  Quoted  by  de  Schweinitz,  Virchow's  Archives.,  xvi.,  233  :  xviii.,  15.  522  :  xx., 
2o5;  xxviii.,  330. 

3  Annales  tf Oculist.,  1861,  xlv.,  199. 


AR  YLARSENA  TE8  279 

Symptoms. — The  symptoms  are  those  of  a  violent  gastro- 
enteritis with  marked  circulatory  depression ;  blindness  comes 
on  from  twenty -four  hours  to  twelve  days  after  the  administra- 
tion of  the  drug. 

Katayama  and  Okamoto  1  collected  twenty-three  cases 
of  poisoning,  of  whom  ten  suffered  from  temporary  or 
permanent  amblyopia  ,  one  or  both  eyes  may  be  involved. 
Stuelp 2  states,  that  of  forty-seven  reported  cases  in  which 
visual  symptoms  were  present,  they  were  noted  as  bilateral 
in  nineteen,  and  as  unilateral  in  seventeen. 

Ophthalmoscopic  reports  vary  a  good  deal ;  some  observers 
have  found  the  fundi  normal  in  cases  of  blindness,  some  simply 
describe  an  optic  atrophy.  Stuelp  examined  a  patient  nine 
hours  after  the  onset  of  coma  from  filix  mas  given  for  anchylo- 
stomiasis.  He  describes  the  whole  fundus  as  covered  with 
white  oedema,  the  arteries  as  thread-like,  the  blood  column 
broken,  the  veins  tortuous  and  filled  with  broad  and  very  dark 
columns  of  blood  ;  blindness  was  complete. 

De  Schweinitz  carried  out  experiements  with  this  drug  on 
dogs  and  rabbits  without  satisfying  himself  that  blindness 
ensued,  nor  did  he  find  any  histological  lesion  in  the  eyes  or 
optic  nerves. 

ARYLARSENATES. 

In  the  early  days  of  the  treatment  of  syphilis  by  the 
arsenic  compounds,  some  of  them  were  found  to  be  especially 
toxic,  and  a  few  cases  of  optic  atrophy  were  reported  from  their 
use.  Two  such  cases  are  reported  by  Clarke 3 ;  Beck  4  has  pub- 
lished twenty-three  cases  of  blindness  from  the  use  of  atoxyl 
in  sleeping  sickness  ;  and  Birch  Hirschfeld  and  Koster  are 
stated  to  have  found  not  only  degeneration  of  the  retina  and 
optic  nerve,  but  also  of  the  external  geniculate  body.  De 
Schweinitz  remarks  upon  the  absence  of  reports  of  visual  dis- 
turbance in  the  numerous  cases  of  arsenical  poisoning,  and 

1  Quoted  by  de  Schweinitz,  "  Toxic  Amblyopias,"  Philadelphia,  1896,  216. 

2  Archiv.  of  Ophth.,  1905,  xxxiv.,  229. 

a  Trans.  Ophth.  Soc.  U.K.,  1910,  xxx.,  240. 

4  Quoted  by  Wray,  Trans.  Ophth.  Soc.,  1910,  xxx.,  24o. 


280  OPTOCHIN 

states  that  he  knows  of  no  reported  cases  of  amblyopia  amongst 
the  arsenic  eaters  of  Styria. 

I  have  seen  changes  develop  in  the  optic  nerve  in  two 
patients,  with  subsequent  serious  defect  of  sight,  whilst  nov- 
arseno-benzol  was  being  administered  for  syphilis,  with  sub- 
sidence of  the  lesions  on  stopping  the  arsenic  compound,  and 
continuing  with  mercury. 

Such  inconclusive  observations,  it  is  clear,  cannot  substantiate 
the  fact ;  at  most  they  suggest  the  possibility  worth  bearing 
in  mind,  that  the  newer  arsenic  compounds  may  not  be  without 
danger  to  the  vision. 

JETHYLHYDROCUPREIN   (OPTOCHIN). 

This  drug  was  first  used  by  Morgenroth  and  Levy,1  in  the 
belief  that  it  possessed  a  specific  action  on  the  pneumococcus. 
In  a  few  cases  of  its  use,  eye  symptoms  have  occurred.  Fraenkel2 
had  three  such  cases  amongst  twenty  patients,  and  John 
Parkinson3  had  one  amongst  nine  patients. 

The  symptoms  in  Fraenkel's  cases  consisted  of  amblyopia, 
which  rapidly  disappeared  on  stopping  the  drug,  and  in  three 
of  Parkinson's  cases  the  pupils  became  widely  dilated,  but  in 
none  of  them  was  amblyopia  developed. 

1  Berlin  klin.  Wochenschr.,  1911,  xlviii.,  ii.,  1560,  1979. 

2  Berlin  klin.  Wochenschr.,  1912,  xlix.,  i.,  664. 

3  Parkinson,  John,  Zeitechr.  f.  Chemotherapie,  1913,  ii.,  1-10. 


CHAPTER   X 

Cysticercus — Echinoeoccufl — Trypanosomiasis — Malaria-^-Filaria  Loa — 
Sporotrichosis — Actinomycosis. 

CYSTICERCUS. 

Many  cases  of  the  localisation  within  the  eye  of  the 
cysticercus  of  Tsenia  Solium  or  Taenia  Mediocanellata  have 
been  recorded. 

According  to  Ballaban,1  who  collected  324  cases  from  the 


FIG.  80. — An  intra-ocular  cysticercus.     (D.  J.  Wood.) 

literature,  the  commonest  site  for  their  occurrence  is  within 
the  vitreous  ;  beneath  the  retina  is  next  most  common,  and 
they  are  least  often  seen  under  the  conjunctiva. 

1   Wien.  Medizin  Wochen.,  1900, 1.,  2018  and  2070. 


282  CYSTICERCUS 

If  the  parasite  is  alive  a  striking  appearance  is  often  seen 
with  the  ophthalmoscope.  A  beautiful,  globular,  translucent 
cyst  may  be  seen,  its  wall  freely  scattered  over  with  fine 
calcareous  granules,  and  movements  of  the  contained  animal 
may  be  visible  (Fig.  80).  Hill  Griffith  1  says  :  "  In  each  case  one 
saw  with  the  ophthalmoscope  a  large  spherical  bluish  white 
cyst,  and  springing  from  this  the  neck  of  the  animal,  like  an 
alabaster  pillar  surmounted  by  the  head  and  suckers,  which, 
with  its  slow,  regular  and  graceful  movements,  reminded  one  of 
an  elephant's  trunk,  the  whole  appearance  presenting  a  picture 
once  seen,  never  to  be  forgotten."  Wood  2  describes  a  rather 
quick  heaving  movement  which  occurred  about  every  fifteen 
seconds,  followed  by  a  slower  subsidence.  He  says  the 
appearance  at  its  acme  was  as  if  something  opened,  and  a 
sharply  defined  semicircle  composed  of  tiny  short  radiating 
lines — evidently  a  part  of  the  hook  circle  at  the  apex  of  the 
animal's  head — appeared  for  a  second  or  less  and  then  subsided 
again  into  the  general  white  mist.  Rembe  3  saw  a  transparent 
cyst  in  the  iris  which  underwent  leech -like  movements. 

Schobl 4  examined  an  eye  histologically  in  which  three 
separate  cysticercus  cysts  were  present. 

If  the  animal  dies,  detachment  of  the  retina  and  disorganisa- 
tion and  shrinking  of  the  eye  ensues.  In  a  few  cases  the  cyst 
has  been  successfully  removed  with  retention  of  good  vision.5 

ECHINOCOCCUS. 

Hyatid  cysts  may  occur  in  the  orbit  or  within  the  eyeball 
itself. 

Intra-ocular. — The  best  description  of  this  exceedingly  rare 
condition  is  given  by  Werner.6  The  eye  of  a  man  aged 
twenty-eight  was  removed  for  secondary  glaucoma.  In  the 
interior  were  a  number  of  spherical  opaque  bodies  which 
proved  to  be  brood  capsules.  Excellent  plates  of  micro  photo - 

1  Trans.  Ophth.  Soc.  U.K.,  1897,  xvii.,  220. 

2  Trans.  Ophth.  Soc.  U.K.,  1901,  xxi.,  89. 

3  Ophth.  Record,  1907,  xvi.,  20. 

4  Central,  fur  prakt.  Augenheil.,  1893,  xvii.,  191. 

5  Gomes,  abstract  Jour.  Amer.  Med.  Ass.,  1919,  Ixxvii.,  1945. 
«  Trans.  Ophth.  Soc.  U.K.,  1903,  xxiii.,  103. 


ECHINOCOCCUS  283 

graphs,  showing  sections  of  the  laminated  ectocyst  and  ruptured 
brood  capsules,  accompany  the  description.  The  cyst  was 
subretinal.  At  the  time  of  Werner's  paper  two  other  cases 
only  had  been  recorded,  one  by  Gescheidt  and  one  by  Hill 
Griffith.1 

Orbital. — Many  cases  of  hydatid  in  the  orbit  are  on  record ; 
they  give  rise  to  a  slowly  developing  exophthalmus,  which 
remains  unchanged  for  a  long  time.  They  are  more  common  in 
the  young  ;  thus  of  thirty-six  cases  collected  by  Lawford,2  two 
only  were  over  forty  years  of  age  ;  the  average  age  was  20-1. 

The  rate  of  growth  seems  to  vary  a  great  deal.  In  a  case 
of  McGillivray,3  exophthalmos  had  existed  for  six  years,  in 
RocklifiVs  4  for  seven  years,  in  Lawson's  5  for  seven-and-a-half 
years,  in  Weeks' 6  for  eight  days. 

TRYPANOSOMIASIS. 

In  this  condition  eye  symptoms  are  common,  and  not  infre- 
quently they  are  the  immediate  cause  of  the  patient  seeking 
advice.  The  most  usual  manifestation  is  an  irido-cyclitis, 
sometimes  with  pronounced  oedema  of  the  lower  lid. 

It  was  pointed  out  by  Daniels  7  that  the  incidence  of  the  eye 
lesions  is  in  direct  relation  with  the  severity  of  the  disease  ; 
thus  in  Rhodesian  trypanosomiasis  the  eyes  were  affected  in 
83-3  per  cent.,  in  Nigerian  cases  40  per  cent.,  and  in  Uganda 
the  incidence  was  only  18-7  per  cent.  ;  the  mortality  of  the 
disease  was  nearly  in  this  ratio.  A  good  description  is  also 
given  by  Jellett.  8 

Some  valuable  experimental  work  was  carried  out  on  dogs 
by  Woods  and  de  Schweinitz.9 

They  found  that  in  these  animals  infected  with  trypanosoma 

1  Trans.  Ophth.  Soc.  U.K.,  1897,  xvii.,  220. 

2  Trans.  Ophth.  Soc.  U.K.,  1895,  xv.,  172. 

3  Quoted  Annal.  d'Oculistigue,  1866,  Ivi.,  174. 

4  Trans.  Ophlh.  Soc.  U.K.,  1889,  ix.,  55. 

5  Soy.  London  Ophth.  Hosp.  Reports,  1882,  x.,  301. 
*  Archiv.  of  Ophth.,  1889,  xviii.,  31. 

7  Proc.  Roy.  Soc.  Med.,  1915-16,  ix.,  iii.,  Ophth.  Sect.,  2. 

8  Ophth.  Review,  1915,  xxxiv.,  41. 

9  Archives  of  Ophth.,  1917,  xlvi.,  431. 


284  FILARIA    LOA 

equiperdum  ocular  complications  were  of  constant  occurrence. 
The  cornea  became  cloudy,  exudate  appeared  in  the  anterior 
chamber,  and  iritis  with  haemorrhages  developed.  These  lesions 
appeared  synchronously  with  the  invasion  of  the  tissues  by 
the  trypanosome  and  seem  to  be  dependent  upon  the  actual 
invasion  and  not  upon  toxaemia.  Trypanosomes  invaded  the 
substantia  propria  of  the  cornea  and  produced  an  interstitial 
keratitis,  and  in  the  iris  and  in  the  focal  masses  of  cellular 
exudate  overlying  the  retina  the  parasite  was  also  present. 

MALARIA. 

There  are  no  distinctive  eye  lesions  of  malaria,  but  an 
occasional  retinal  haemorrhage  is  seen,  and  it  has  been  pointed 
out  by  Kipp  l  that  dendritic  corneal  ulcers  are  more  frequent  in 
this  than  in  other  febrile  diseases. 

FILARIA   LOA. 

The  geographical  distribution  of  this  worm  is  limited  to 
Congoland  and  Old  Calabar  on  the  West  Coast  of  Africa. 

It  may  appear  under  the  integument  in  any  part  of  the  body, 
but  with  especial  frequency  under  the  skin  of  the  eyelids  or 
under  the  conjunctiva  ;  never  within  the  eyeball.  According  to 
Sir  Patrick  Manson  it  varies  in  length  from  17  to  70  mm.,  the 
male  being  the  smaller. 

The  parasite  manifests  itself  by  causing  local  irritation,  and 
may  be  seen  moving  rapidly  under  the  skin  or  conjunctiva. 

Argyll  Robertson  2  says  "it  is  capable  of  wandering  at  will 
from  one  eye  to  the  other,  to  disappear  into  the  deeper  parts 
of  the  orbit,  to  wander  under  the  skin  of  the  eyelids,  and  even 
to  make  excursions  into  the  temporal  region  "  ,  after  removal, 
the  worm  presents  the  appearance  of  "  a  piece  of  fishing  gut, 
being  round,  firm,  transparent  and  colourless." 

During  cold  weather  it  retires  to  the  deeper  tissues  and 
causes  no  symptoms,  coming  towards  the  surface  and  appearing 

1  Tram.  Amer.  Ophth.  Hoc.,  1880-84  iii.,  91 ;  and  1880-90.  v.,  331. 

2  Trans.  Ophth.  Soc.  U.K.,  1895,  xv.,  137. 


8POROTRICHOSIS  285 

under  the  skin  or  conjunctiva  in  warm  weather  or  in  a  warm 
room. 

If  it  is  required  to  remove  the  parasite,  its  movements  in  the 
tissues  are  so  quick  that  it  is  necessary  to  grip  it  through  the 
tissues  with  forceps  or  to  under-run  it  with  a  silk  stitch,  so  as 
to  fix  it  before  making  an  incision. 

In  a  case  reported  by  Thomas  and  Parsons  l  a  small  round 
segmented  worm  12  mm.  long  was  seen  lying  on  the  iris.  The 
eye  was  removed,  and  the  head  of  the  larva  was  submitted 
to  Sir  Arthur  Shipley,  who  pronounced  it  to  be  either  the 
maggot  of  a  blow-fly  or  a  Sarcophaga  Carnaria. 


SPOROTRICHOSIS. 

A  few  cases  of  infection  of  the  conjunctiva  by  the  Sporo- 
trichum  Beurmanni  have  occurred. 

The  palpebral  conjunctiva,  the  plica  semilunaris  and  the 
lachrymal  sac  are  most  affected.  Nodules  appear  which  are 
usually  small,  but  are  sometimes  of  considerable  size  ;  Chaillous  2 
describes  some  as  being  as  large  as  a  bean.  These  nodules 
break  down  to  form  greyish  yellow  ulcers  with  the  formation 
of  a  good  deal  of  pus.  The  preauricular,  submaxillary  and 
cervical  lymphatic  glands  become  much  enlarged,  and  fever 
may  occur. 

It  is  a  condition  which  is  difficult  of  cure  ;  thus  Bedell  3 
records  a  case  of  two  years'  duration,  and  Chaillous  one  of  more 
than  a  year.  Wilder  and  McCullough  4  report  a  case  which 
occurred  in  a  student  engaged  in  investigating  the  condition 
which  was  completely  cured  in  two  months.  In  Bedell's  case, 
several  firm  concretions  were  removed  from  the  lachrymal  sac 
from  which  the  sporothrix  was  obtained  on  culture  after 
crushing  ;  Morax  5  also  found  it  present  in  the  lachrymal  sac. 

1  Trans.  Ophth.  Soc.  U.K.,  1909,  xxix.,  14. 

2  Annales  d'Ocul..  1911,  cxlv.,47. 

3  Trans.  Amer.  Ophth.  Soc.,  1912-14,  xiii.,  720. 

4  Jour.  Amer.  Med.  Assoc.,  1914,  Ixii.,  1156. 
3  Annales  tfOculistique,  1911,  cxlv.,  49. 


286  ACTINOMYCOSIS 

ACTINOMYCOSIS. 

Cases  of  external  infection  of  the  eye  by  actinomyces  have 
been  recorded,  usually  as  a  result  of  trauma. 

The  fungus  was  found  by  Orloff  in  two  cases  of  corneal  ulcer 
six  weeks  after  an  injury.  Rosenhauch x  reports  a  similar 
case  following  injury  by  a  thorn,  and  also  a  case  in  which  the 
conjunctiva  was  apparently  infected  by  a  coriander  seed  which 
had  lodged  in  the  sac. 

In  a  case  of  miliary  actinomycosis,  Miiller  2  found  numerous 
nodules  in  the  retina  around  the  disc,  which  on  examination 
were  found  to  be  composed  of  large  epithelioid  cells  with  poorly 
staining  nuclei. 

1  Klin.  Monats.f.  Augen.,  1913,  xv.,  818. 

2  Klin.  Monats.  f.  Augen.,  1903,  xli.,  230. 


INDEX 


Abortitacient,  quinine  as,  267 
Acromegaly,  accompanying  defects  of    visual 
fields    complicating  'diseases    of   pituitary 
bodies,  1'jo 

Actinomyces,  external  infection  of  eye  by,  286 
Adrenalin,    instillation    of,    effect  'on    pupil, 

in  injury  to  sympathetic  nerve,  95 
yEthylhydrocuprein  (optochin),  administration 

of,  eye  symptoms  following,  280 
Albinism  and  nystagmus,  26,  27 
Albuminuria  in  thrombosis  of  retinal  veins,  85, 

86 

Alcohol-tobacco-amblyopia,  258,  262 
Alkaptonuria,  ochronosis  of  sclerotic  in,  277 
Amaurosis,  cerebral  acute,  of  infancy,  249 

fvgax,      complicating       retinal      arterio- 
sclerosis, 63 
of    post-basic    meningitis,    retention    of 

pupillary  light  reflex  in,  40 
puerperal,    retention    of    pupillary    light 

reflex  in,  40 
uraemie,  158 
without  ophthalmoscopic  signs  in  cerebro- 

spinal  meningitis  in  children,  203 
Amaurotic  family  idiocy,  217 

changes  in  central  nervous  system  in,  218, 

219 

in  ganglion  cells  of  retina  in,  219,  220 
cherry-red  spot  at  macula  in,  217,  220 
clinical  stages  of,  217,  218 
limited  to  Jewish  children,  217 
Amblyopia,  alcoholic,  261 

histological  changes  in,  263,  264 

incidence,  262 

Leber's  disease  diagnosed  as,  262 

signs  and  symptoms,  263 

whiteness  of  temporal  half  of  papilla  in,  263 

See  also  Alcohol-tobacco-amblyopia. 
Amblyopia  due  to  bilateral  ocular  defects  in 

very  early  life,  28 
due  to  iodoform  poisoning,  274 
electric,  203,  254 
following  administration   of  sethylhydro- 

cuprein  (optochin),  2wO 
following  poisoning    by    aspidium  (Filix 

mas),  279 

hysterical,  98,  99,  100 
in  lead  poisoning,  272 

See  also  Tobacco  amblyopia. 
Ansemia,  pernicious,  retinal  haemorrhages  in, 

69 
Anaemias,  secondary,  fund  us  changes  in,  76,  77 

retinal  haemorrhages  in,  76,  77 
Anaesthesia,  spinal,  indirect  paralysis  of  cranial 

nerves  following,  42,  43 
Aneurysms  of  large  arteries  and  veins  of  retina, 

230 
Angio-sclerosis,      producing     thrombosis      of 

retinal  veins,  85 
Aniline  dye  workers,  pigmentation  of  cornea 

and  conjunctiva  in,  276 
Anterior    chamber,    exudate    in,    in    primary 

gonococcal  iritis,  208,  209 
Aqueous  humour,  increase  in  salts  of  following 

administration  of  naphthalin,  277 
Area  striata,  1,  2 

Argyll  Robertson  pupil,  syphilitic  origin  of,  114 
Arteries,    retinal,    as    guide    to   condition   of 

cerebral  arteries,  59 

irregularity  of  lumen  in  'arterio-sclerosis, 
~ 


Arterio-sclerosis,  50 

producing  thrombosis  of  retinal  veins,  85 
retinal,  51 

amount  of  blood -pressure  in,  66 

and  glaucoma,  no  direct  relationship 

between,  68 
appearance  of  central  light  streak  in, 

51 
association  with  vascular  lesions  of 

brain,  59,  60 
complications,  63 
discovery  by  ophthalmoscopy,  .">0 
evolution  of  arterio-sclerotic  retinitis 

from,  57 

retinal  haemorrhages  in,  55 
optic  atrophy  in,  67 
prognosis,  62,  63 
sex-incidence,  63 
sluggish  pupil  reaction  in,  55 
tortuosity  of  arteries  in,  54 

of    small    vessels    in    region    of 

macula  in,  54,  55 
Arterio-venous  crossings,  51,  52 
Artery  and  veins,  retinal,  thrombosis  of,  com- 
plicating facial  erysipelas,  207 
cerebral,  right  posterior, 

obstruction  of  position  of  scotoma  in, 

6,7 
thrombosis  of,  visual  fields  in  case  of, 

diagrams  showing,  6,  7 
ophthalmic,   obstruction  due  to  tumour 

pressure  at  optic  foramen,  16 
retinal,  central, 

embolism  of,  198 
site  of  arterio-sclerosis  in,  54 
branches  in  retina,  constrictions    of 

lumen  in  arterio-sclerosis,  54 
embolism  in,  followed  by  panophthal- 

mitis,  207 

pipe-stem  sheathing  of,  59 
right,  embolism  in  branch  of,  com- 
plicating    infective     endocarditis, 
205,  206 

thrombosis  of,  66 

vein,  and  lymphatics,  retinal    blood-pres- 
sure in,  compared,  16 
Arthritis,    associated    with   eye   affections   iu 

dysentery,  209,  210 
gonococcal,  date  of  development  of  iritis 

after  onset  of,  208 
recurrences  of,  208 

Arylarsenates,   administration,   ocular   lesions 
and  visual  disturbances  following,  279,  280 
Aspidium  (Filix  mas),  poisoning  by,  278 
followed  by  amblyopia,  279 
ophthalmoscopic  appearances  after,  279 
symptoms,  279 
Ataxia  and  optic  atrophy,  partial  dissociation 

118 
Friedreich's,  eye  symptoms  in,  120 

nystagmus  in.  12(1 
relation  of  optic  atrophy  to,  117 
Atheroma  of  great  vessels,  in  relation  to  retinal 

arteries,  61 
Atoxyl,  use  of,  in  sleeping  sickness,  followed  by 

blindness,  279 
ocular  lesions  following,  279 

Barlow's  disease.     See  Scurvy,  infantile. 
Biscuit  diet,   exclusive,    producing   condition 
resembling  kerato-malacia  iu  dog,  189 


288 


Bisulphide  of  carbon  poisoning,  274 

committee  of  investigation  into,  275 
path  of  entrance  into  body,  27-~> 
symptoms,  275 
visual  disturbances  in,  275 
Blepharospaam,  development   of,   in    soldiers 

during  war,  101 
Blindness  due  to  facial  erysipelas,  207 

following   division   of   optic    nerve   from 
fracture  involving  anterior  cranial 
fossa,  48 
leprosy,  212 

non-traumatic  haemorrhages,  78,  79 
use    ofatoxyl    in   sleeping  sickness. 

279 

hysterical,  method  for  curing,  100 
in  horses  in  New  South  Wales,  260 
permanent,  following  thrombosis  of  retinal 

artery,  66 
in  acute  cerebral  amaurosis  of  infancy, 

250 

retention  of  pupillary  light  reflex  in,  40 
temporary,     during     spasm     of     retinal 

arteries,  65 
total,  from  overdoses  of  sodium  salicylate, 

270 

temporary,  following  antiseptic  appli- 
cation of  phenol,  276 
ursemic,  retention  of  pupillary  light  reflex 

in,  40 
See    Quinine    blindness,  Wood    alcohol 

blindness. 
Blood  entering  subarachnoid    space,   further 

passage  of,  46 

Blood-pressure,  amount  of,  in  retinal  arterio- 
sclerosis, 66 

and  intra-ocular  pressure,  68 
heightened  in  diabetic  retinitis,  161,  163 
in  retinal  artery,  vein  and  lymphatics,  16 
in  thrombosis  of  retinal  vein,  85 
increase  in  renal  retinitis,  136 
Blood-vessels,  cerebral,  third  nerve  connected 

with,  43 
great,  atheroma  of  in  relation  to  retinal 

arteries,  61 

of  face  and   neck,  constriction  of,  in  irrita- 
tion of  sympathetic  nerve,  96 
retinal,  appearances  at  death,  48,  4!) 

engorgement  and  distension    in    leu- 
kaemia, 70,  71 

engorgement  in  cyanosis  retinae,  83 
minute  examination  under  ophthal- 
moscope, 50 

spasm  in  Kaynaud's  disease,  78 
Blue  sclerptics,  223 

associated  with  brittle  bones,  224 
pathology,  22.~> 
pedigrees  of,  224 
Boys,    greater   prevalence   of   word    blindness 

among,  221,  222 

Brain,  abscess  of,  papilloedema  with,  21 
area  striata  of,  1,  2 
cortex  of,  portion  connected  with  central 

vision,  1,  6 
cortical  areas  of,  distribution,  diagrams 

illustrating,  2,  3 
destruction  by  softening  or  clot,  rarity  of 

optic  neuritis  in,  19 

disease  of,  paralysis  of  ocular  nerves  un- 
certain value  as  localising  sign  in,  105 
gunshot  injuries  of, 

investigation  of  visual  field  for  appre- 
ciation of  movement  after,  11 
correlation  with  visual  fields,  4,  5 
recovery  of  vision  after,  10,  11 
lesion,  site  of,  in  disturbances  of  visual 

orientation,  94 
lesions  of,  accompanied  by  disturbances  of 

visual  orientation,  93 
softening  of,  rarity  of  papilloedema  in,  19 
tumour  of,  counterfeited  by  symptoms  of 

chlorosis  with  disc  changes,  75 
incidence    of    papilloedema    varying 
with  site  of,  18 


Brain,  tumour  of,  producing  optic  atrophy  in 

one  eye,  and  papilloeduma  in  other,  21 
tumours  of,   length    of    time    for    which 
present  before  onset  of  papilloedema, 

19,  20 

papilloedema  in,  17,  149,  150 
recurrence     without     recurrence     of 

papilloedema,  19 
vascular  lesions  of,  retinal  arterio-sclerosis 

associated  with,  59,  60 
Brittle   bones,   associated  with  blue  sclerotics, 


Calcarine    fissure,   posterior,  representation  of 

upper  parts  of  retina  in,  8,  9 
wounds  of  upper  lip  of,  visual  fields 

affected  by,  8,  '.» 
softening  at  bottom  of,  position  of  scotoma 

following,  10 
Carbolic  acid,  antiseptic  use  of  followed   by 

emporary  total  blindness,  276 
Cataract,     double,     following     experimental 

administration  of  naphthalin,  277 
diabetic,  characteristics  of,  169,  170 

removal,  170 
in  Mongolian  idiocy,  249 
in  myotonia  atrophica,  130 
Cavernous  sinus,  thrombosis  of,  '.)(i 

accompanied   by  chemosis  and  oedema  of 

lids,  91 

by  oedema  of  retina,  91 
by  proptosis,  91 
by  swelling  of  disc,  91 
by  tortuous  veins,  91 
causes  of,  90 

changes  in  eye  due  to  development  of,  91 
due  to  otitis  media,  92 
frequency,  91 
infective,  90,  92 

involvement  of  ocular  nerves  in,  91. 
traumatic,  90,  92 
Cellulitis,  orbital,  distinction  of  cavernous  sinus 

thrombosis  from,  91 
following  facial  erysipelas,  207 
Cerebellum,      lesions     of,      associated      with 

nystagmus,  31,  32 

tumours  of,  severity  of  papilloBdema  in,  18 
Cerebral    decompression    operations    for    pre- 
servation of  sight  in  papilUedema,  21,  22 
Chancre,  primary,  of  eyelid,  190 

of  lachrymal  sac,  190 
Cherry-red  spot  at  macula  in  amaurotlc  family 

idiocy,  217,  220 

Chiasma,  optic,  degenerative  changes  in  dis- 
seminate sclerosis,  127 

Chiasmal  cross-roads,  distortion  by  growth  in 
interpeduncular  space,  without  disturbance 
of  vision,  185 

Children     with      cerebro-spinal       meningitis, 
amaurosis    without    ophttialmoscopic    signs 
in,  203 
Chloroma,  73,  74 

association  with  leukaemia,  74 
dissemination  into  retina  and  choroid  of 

one  eye  in  case  .of,  75 
green  deposits   in   periosteum  of  lumbar 

vertebrae  in,  74 

greenish  thickening  of  skull  bones  in,  74 
infiltration  of  tissues  with  myelocytes  in, 

74 

pathology  of,  74 
proptosis  in,  74 
Chlorosis,   papilloedema     and     pseudo-papill- 

oedema  in,  75 

pathological  changes  in  fundus  oculi  in,  75 
retinal  haemorrhages  in,  75 
star  figure  at  macula  in,  75 
symptoms  of,  with  disc  changes,  counter- 
feiting cerebral  tumour,  75 
Choked  disc,  12 

and  optic  neuritis,  distinction  between,'17 
in  diseases  of  pituitary  body,  186 
trephining  in,  22 


INDEX 


289 


Chorio-retinitis,  syphilitic,  194 

night  blindness  in,  223 

Choroid    and   retina   of   one  eye,  dissemina- 
tion of  chloroma  into,  75 
anterior  part,  infection  in  leprosy,  213 
blood  vessels,  sclerosis  of,  61 
engorgement  with  blood  in  case  of  ery- 

thrsemia,  81,  82 

infiltration  of,  in  leukaemia,  section  show- 
ing, 72 

supposed  tuberculous  lesions  of,  198 
tubercle    in,    from    case    of    tuberculous 

meningitis,  section  through,  200 
tubercles  in,  in  miliary  tuberculosis,  198 
without    invasion    of    meninges, 

HI'.),  200,  201 
in  tuberculous  meningitis,  198 

unilateral,  199 
Choroiditis,  syphilitic,  193,  194 

congftiital,  194 
Ciliary,  body  and  iris,  gross  tuberculous  disease 

of,  197 
spontaneous   bleeding  from,   in  leu- 

k:emia,  72 

body,  infection  of  in  leprosy,  213 
muscle,  paralysis  of,  following  diphtheria, 

Colour,  losses  of  visual  fields  for,  11 
blindness  in  lead  poisoning,  272 
perception,  loss  of,  preceding  that  for  form 
in  irregular  defects  of  visual  fields 
accompanying  pituitary  lesions,  182 
probable  centre  for,  11 
sensations,  subjective,  in  lead  poisoning, 

vision,  impairment  of  in  tabes,  120 
Conjunctiva,  effect  of  nitrate  of  silver  on,  273 
leuka-mic  tumours  developing  under,  73 
skin  of,  filaria  loa  under,  284 
sporotrichosis  of,  285 
xerosis  of,  night-blindness  associated  with, 

223 
Conjunctivitis,  diphtheritic,  211 

fictitious,  due  to  placing  soap  in  eyes,  100 
gonorrhoea!,  metastatic,  209 
in  cerebro-spinal  meningitis,  202 
in  dysentery,  209,  210 
recurrent,  associated  with  gout,  160 
"  Copper-wire  "  arteries,  51,  54 
Cornea,  pigmentation  in  aniline  dye  workers,  276 
Corpora     quadrigemina,     lesion     of,     causing 
ophthalmoplegia  with  inco-ordination  of 
lower  limbs,  108 
ocular  symptoms   of  involvement  of,   in 

third  nerve  paralysis,  108 
superior  development  in  birds  and  reptiles, 

41 

relation  to  vision,  41 
Cotton-wool  patches  on  retina  in  renal  retinitis, 

140,  141,  154,  155 

Cranial  fossa,  anterior,  fracture  involving,  caus- 
ing division  of  optic  nerve,  48 
nerve  palsies  in  encephalitis  lethargica,  114 
nerves,  indirect  paralysis  of,  41 

following  spinal  anaesthesia,  42,  43 
sinuses,  thrombosis  in  erythrsemia,  82 
Crystalline  lens,  dislocation  in  executions  by 

hanging,  49 
Cyanosis  retina?,  82,  83 

engorgement  of  retinal  blood  vessels  in,  83 
retinal  haemorrhages  in,  83 
Cycloplegia  in  tabes  and  general  paralysis,  116 
Cysticercus,  localisation  within  eye, 
ophthalmoscopic  appearances,  282 
ocular  lesions  following  death  of  parasite, 

282 

Cytoid  bodies,  formation  of,  in  leukaemic  reti- 
nitis, 73 

Dalrymple's  sign  in  exophthalmic  goitre,  172, 

Davy  lamp,  use  of,  favouring  development  of 
miner's  nystagmus,  29,  30 
M.O. 


Death,  appearances  in  retina  at,  48,  49 
Diabetes,  alterations  of  refraction  in,  169 
cataract  in,  169,  170 
intra-ocular  haemorrhages  in,  165 

statistics,  167 
iritis  in,  167 

lipsemia  retinalis  in,  167,  163 
ocular  tension  in,  172 

See  aim  Ketinitis,  diabetic. 
Diabetics,  retinal  haemorrhages  in,  165,  166 
optic  atrophy  in,  171 
retro-bulbar  neuritis  in,  170 
toxic  effect  of  smoking  in,  171 
Dilator  pupillae,  nerve  fibres  controlling,  94 
Diphtheria,  ocular  complications  of,  211,  212 
Diplopia,  accompanying    paralysis  of  external 

muscles  of  eyes,  104 
early    prodromal    sign    of    encephalitis 

lethargica,  112 
following    post-diphtheritic    paralysis    of 

extrinsic  ocular  muscles,  212 
in  disseminate  sclerosis,  125 
in  myasthenia  gravis,  130 
intermittent,  accompanying  insular  scler- 
osis and  myasthenia  gravis,  105 
limitation  of  in  field  of  vision  in  paralysis 

of  external  ocular  muscles,  105 
monocular,  44 

accompanying     paralysis     of     sixth 

nerve,  44,  45 
temporary  occurrence  of  in  Arctic  regions, 

251 

Disc,  optic,  appearances  in  papilloedema,  17 
changes  in  disseminate  sclerosis,  123,  124 
resulting  from  syphilis,  194,  195 
with  symptoms  of  chlorosis,  counter- 
feiting cerebral  tumour,  75 
congested  appearance  in  retinal  arterio- 
sclerosis, 55 

development  of  tortuous  veins  on,  after 
recovery  from  thrombosis  of  retinal 
veins,  87,  88 

oedema  of,  in  tuberculous  meningitis,  201 
ophthalmoscopic  appearances  in  papilto- 

dema,  14,  15 

pallor  of,  in  tobacco-amblyopia,  260 
swelling  of,  accompanying  cavernous  sinus 

thrombosis,  91 

Discs,     optic,     ophthalmoscopic     appearance 
similar  in  Leber's  disease  and  pituitary  dis- 
turbance, 217 
Dropsy,    epidemic,    development    of    chronic 

glaucoma  in,  68 
Dysentery,  eye  affections  in,  209 

associated  with  arthritis,  209,  210 


Kales'  disease.    See  Haemorrhage,  intra-ocular, 

recurrent. 
Echinococcus,  intra-ocular,  282 

orbital,  283 

Eclampsia  with  visual  disturbances,  138 
Eclipse  blindness,  252 

prognosis,  253 
Electric  flashes,  intense,  resulting  in  blindness, 

252 

Embolism  in  branch  of  right  retina}  artery  com- 
plicating infective  endocarditis,  205,  206 
of  central  retinal  artery,  198 
of  retinal  artery,  followed  by  panophthal- 

mitis,  207 
Encephalitis  lethargica,  111 

ocular  symptoms  of,  112 
Endocarditis,  infective,  complicated 

by  embolism  of  branch  of  right   retinal 

artery,  205,  206 

by  retinal  haemorrhages,  205,  206 
Epilepsy  and   migraine,  supposed  affinity  be- 
tween, 239 

subjective  ocular  symptoms  in,  101 
visual  warning  of,  102 

Equilibrium  of  body,  conditions  disturbing, 
favouring  development  of  miner's  nystag- 
mus, 30 

19 


290 


INDEX 


T!r\>i]M'l:is,  facial,  causing  blindness,  207 

complicated  by  thrombosis  of  retinal  artery 

and  veins,  207 

followed  by  orbital  cellulitis,  207 
Erythraemia  (Vaquez's  disease),  80 
age  and  sex  incidence,  80 
engorgement  of  choroid  with  blood  in,  81 
of  retinal   veins    with    haemorrhages 

in,  82 
loss  of  vision  with  slight  ophthalmoscopic 

changes  in,  80,  81 
ophthalmoscopic  changes  in,  82 
thrombosis  of  cranial  sinuses  in,  82 
visual  symptoms  in,  82 
Exophthalmos  associated  with  uraemia  in  renal 

retinitis,  139 

in  diseases  of  pituitary  body,  187 
in  exophthalmic  goitre,  173,  174,  175,  176 
continuance  of  after  cure  of  goitre,  174 
loss  of  both  eyes  in,  174 
in  pxycephaly,  234,  235 
in  irritation  of  sympathetic  nerve,  96 
Exudates,  retinal,  in  arterio-sclerotic  retinitis, 

55 

in  renal  retinitis,  140 
Eyelid,  primary  chancre  of,  190 
Eyelids,  chemosis  and  oedema  of,  accompanying 

cavernous  sinus  thrombosis,  91 
ecchymosis  of,  in  infantile  scurvy,  188 
margins  of,  invaded  by  leprosy  bacilli,'  213 
external  infection  of,  by  actinomyces,  286 
skin  of,  fllaria  loa  under,  284 
Eye  movements  in  nystagmus,  rate  of,  24 
Eyes,   hypermetrophic,    tortuosity  of    retinal 

vessels  in,  230 

upward  movement  of,  paralysis  of  in 
association  with  paralysis  of  le vat ors, 
108 

Fan-shaped  figure  in  retina  in  papilloedema,  147 
Fat,  formation  in  retina,  in  renal  retinitis,  142, 

143 
increase  of  in  orbit,  causing   proptosis  in 

exophthalmic  goitre,  175,  176 
Fat-soluble    vitamin,     absence,     of,     in     diet 

causing  kerato-malacia,  188, 189 
Feeding,    improper,    causing    kerato-malacia, 

188,  18!) 

Fifth  nerve,  paralysis,  in  cerebral  syphilis,  196 
Filaria  loa,  removal  of,  285 

under  skin  of  eyelids  or  conjunctiva,  284 
"  Fistel-symptom  "  in  relation  to  production 

of  labyrinthine  nystagmus.  33 
Fixation  point,  position  of  scotoma  in  relation 

to,  6,  7 

Fourth  nerve,  paralysis  in  cerebral  syphilis,  196 
Fractures,  multiple,"  of  brittle  bones  associated 

with  blue  sclerotics,  m 
Frontal  bones,  greenish  thickening  in  chloroma, 

Fundus  oculi,  appearances  after  death,  48 

appearances      of,     after      poisoning      by 

Aspidium  (Filix  mas),  279 
changes  in,  in  secondary  anaemias,  76 
colour  of,  in  nitro-benzol  poisoning,  276 
pathological  changes  in,  in  chlorosis,  75 
white  patches  on,  in  secondary  anaemias,  77 

Geniculate    body,    external,    degeneration    of 

following  use  of  atoxyl,  279 
Gennari,  stria  of,  1,  2,  3 
Glaucoma, 

chronic,  developing  in  epidemic  dropsy,  68 
and    retinal    arterio-sclerosis,    no    direct 

relationship  between,  68 
development    in    thrombosis    of    central 

retinal  vein,  68,  87 
Goitre,  exophthalmic,  aetiology  discussed,  176, 

Dalrymple's  sign  in,  172, 177 

defective  ocular  movements  in,  177.  178 

exophthalmos  in,  173 

von  Graefe's  sign  in,  172 

Moebius'  sign  in,  173 


Goitre,  retro-lmlbar  neuritis  in,  170 

Stellwag's  sign  in,  173 
Gonococcus.     See  Iritis,  gonocon  us. 
Gonorrhoea,  endogenous,  20!> 
Gout,  ocular  lesions  related  to,  I.V.I 
Graefe's  (von)  sign  in  chronic  nephritis,  139 

in  exophthalmic  goitre,  172 
Gunshot  injuries  of  brain,  investigation  of  visual 
field     for    appreciation    of    movement 
after,  11 

injury  to  occipital  cortex  and  optic  radia- 
tions,   inferior    hemianopia    following, 
perimeter  charts  showing.  8,  9 
wounds   of  brain,  correlation  with  visual 

tidds,  4,  5 

recovery   of   vision  after,   10,  11 
upper  lip  of  posterior  calcarine  fissure, 
visual  fields  affected  by,  8,  9 


Haemophilia,  absence  of   retinal  haemorrhages 

in,  77 
Haemorrhage,  cerebral,  associated  with  retinal 

arterio-sclerosis,  59,  60 
or  intra-cranial,  rarity  of  papilloedema 

in,  19 

intestinal,  followed  by  blindness,  78 
traumatic,  rarely  followed  by  blindness,  78 
in  yellow  spot  region,  35 
into  optic  nerve  sheath,  45,  46 

following  fracture  of  skull,  46,  47 
intra -ocular  recurrent,  causes,  243 
in  young  adults,  242 
loss  of  sight  in,  244 
ophthalmoscopic  features,  242 
symptoms.  24:! 
retinal,   accompanying   haemorrhage   into 

optic  nerve  sheath,  47 
uterine,  followed  by  blindness,  78 
Haemorrhages  in  papillo-dema,  15 

into  nerve  fibre  layer  of  retina,  34,  35 
intra-ocular,  in  diabetes,  165 
large,  non-traumatic,  followed  by  blind- 
ness, 78,  79 
retinal,  34 

absence  in  haemophilia,  77 
absorption,  37 
colour  of,  35,  36,  37 
flame-shaped,  35,  36 
in  chlorosis,  75 
in  cyanosis  retinae,  83 
in  diabetes,  165,  166 
in  erythru'mia,  82,  83 
in  leukaemia,  71 
in  malaria,  284 
in  new-born  infants,  83,  84 
in  pernicious  anaemia,  69 
in  purpura,  77 

in  pya-mia  and  septicaemia,  204 
in  Kaynaud's  disease,  78 
in  renal  retinitis,  140 
in  retinal  arterio-sclerosis,  55 
in  secondary  anaemias,  76,  77 
in  thrombosis  of  retinal  veins,  86 
prognosis,  62 

time   required   for  disappearance  of 
different  types  of,  composite  dia- 
gram showing,  36 
Headache,   severe,    associated   with   recurrent 

temporary  ocular  palsies,  240 
Head  nodding  and  nystagmus  (Upaxmits  nutatis), 

33 

Hemianopia  accompanying  migraine,  239 
bi-nasal,  39,  40 

complicating  tabes,  40 
location  in  optic  nerve,  40 
concomitant  of  migraine,  241 
bi-temporal,  in  lesions  of  pituitary  body, 

180,  181 
in  tabes,  119 

due  to  obstruction  of  right  posterior  cere- 
bral artery,  position  of  scotoma  in,  6,  7 
homonymous,  in  lesions  of  pituitary  body, 
180   181 


INDEX 


291 


Hemianopia  in  retrobulbar  neuritis,  100 

inferior,  nearly  complete,  from  gunshot 
injury  to  occipital  cortex  and  optic 
radiations,  perimeter  charts  showing,  8, 
9 

permanent,  resulting  from  migraine,  241 
quadrantic,    lesions    of    optic    radiations 

associated  with,  10 

temporary,  accompanying  migraine,  course 
of  dividing  line  between  seeing  and 
blind  halves,  6 

Hemiatrophy,  slight,  in  ocular  torticollis,  227 
Hemiplegia,  crossed,  bilateral  third  nerve  para- 
lysis in  cerebral  syphilis  with,  196 
unilateral  and  bilateral  third  nerve  para- 
lysis in  cerebral  syphilis  without,  196 
Heredity  in  congenital  night  blindness,  222 
in  Leber's  disease,  214 
in  nystagmus,  26,  27 
Herpes  dphthalmicus,  244 
effect  on  cornea,  246 
form  of  eye  affection  in,  245 
pathology,  246 
severity  of  in  relation  to  herpes  zoster  of 

nose,"  24."> 

Horses  in  New  South  Wales,  blindness  in,  260 
Hot  eye,  160 
Hydrocephalus,    defects    of    vision    resulting 

from  primary  optic  atrophy,  111 
Hypothalamic  region,  sympathetic  centre  in,  94 
Hysteria,    idiopathic,    occurring    usually    in 

females,  98 

onset  of  symptoms  insidious  in,  98 
loss  of  sight  in",  98 
ocular  manifestations  of,  98,  101 
traumatic,  98 

Infants,  kerato-malacia  in,  188 

new-born,  retinal  haemorrhages  in,  83 
Interpeduncular  space,  growth  in,  causing  dis- 
tortion   of    chiasmal    cross-roads,    without 
disturbance  of  vision,  185 
Intestinal   tract,    bleeding   from   followed    by 

blindness,  78 
Intra-cranial  pressure,  increase  in,  in  papillce- 

dema,  15,  16 

increase  of,  protection  of  retinal 
vessels  against  in  case  of  tumour  at 
optic  foramen,  16 

tumour  accompanied  by  nystagmus,  31 
tumours,    indirect     paralysis    of    cranial 

nerves  accompanying,  42 
Intra-ocular  pressure 

and    blood-pressure,  connection    between 

denied,  63 

temporary  reduction,  means  for,  68 
lodoform  poisoning,  amblyopia  due  to,  274 
Irido-cyclitis  in  dysentery,  209 
in'trypanosomiasis,  283 
tuberculous,  severe,  197 
Iris  and  ciliary  body, 

gross  tuberculous  disease  of,  197 
spontaneous    bleeding   from   in   leu- 
kemia, 72 
condition  of,  in  paralysis  of  sympathetic 

nerve,  96 

Iritis,  forms  of,  complicating  leprosy,  213 
gonococcal,  207,  208 

date  of  development  after  onset  of 

iritis,  208 
primary,    with    exudate    in    anterior 

chamber,  208,  209 
rarity  in  women,  208 
recurrences  of,  208 
gouty,  160 
in  diabetes,  167 
syphilitic,  192 

congenital,  192 
time  of  onset,  193 
with  keratitis  in  herpes  ophthalmicus,  245 

Jaw  blinking  in  infants  and  children,  227 
ptosis  in,  227 


Keratitis,  interstitial,  191 

complicating  leprosy.  213 

due  to  congenital  syphilis,  191 

onset    and    course    unhindered    by 

treatment,  193 
rarely  following  acquired  syphilis,  191 

192 
unilateral,  developing  in  eye  site  of 

primary  chancre,  190 
neuroparalytic,  254 

a?tiology  discussed,  255 
oyster  shucker's,  256 
with  iritis  in  herpes  ophthalmicus,  245 
Kerato-malacia  in  infants,  188,  189 
Kidney  disease, 

papillcedema  with,  trephining  contra- 
indicated  in,  23 
retinitis  in,  131 

Set  alto  Retinitis,  renal, 
small  white,  retinal  changes  in,  156 

Lachrymal  glands,  leukaemic  tumours  develop- 
ing in,  73 

sac,  primary  chancre  of,  190 
Lactation,  prolonged,  loss  of  sight  following, 

248 
Lagophthalmos,  in  anaesthetic  leprosy,  213 

in  myasthenia  gravis,  130 
Lardaceous  disease,  ocular  symptoms  generally 

absent  in,  159 
Lead  poisoning,  270 

loss  of  vision  in,  272 

ocular    symptoms    and    lesions    accom- 
panying, 271,  272 

Leber's  disease,  age  and  sex  incidence,  214,  215 
central  scptoma  of,  215 
characteristics  and  course  of,  214,  215 
diagnosed  as  alcoholic  amblyopia,  262 
heredity  in,  214 

impairment  of  vision  in,  214,  215 
in  relation  to  disturbances  of  pituitary 

body,  216,  217 
recovery  of  vision  in,  215 
Lens  opacities  resulting  from  lightning,  254 
Leontiasis  ossea,  diagnosis,  236 

ocular  manifestations  in,  236 
Leprosy,  followed  by  blindness,  212 

infection  of,  means  of  conveyance  to  eye, 

213 
ocular  complications  of,  212 

clinical  manifestations,  213 
bacilli  invading  margins  of  eyelids,  213 
Leukaemia,  70 

association  of  chloroma  with,  74 
engorgement    and    distension    of    retinal 

blood  vessels  in,  70,  71 
lymphocytic,  incidence,  70 
myelogenic,  incidence,  70 
op'hthalmoscopic  changes  in,  70 
retinal  haemorrhages  in,  71 
sex  incidence,  70 
Leukaemic  infiltrations  of  orbit,  73 

tumour  developing  in  lachrymal  glands,  73 

tumours  developing  under  conjunctiva,  73 

Levators,    paralysis    of,    in     association    with 

paralysis  of  upward  movement  of  eyes,  103 
Light  reflex,  pupillary,  retention  in  cases  of 

blindness,  40 

Lightning,  eyes  affected  by,  254 
Limbs,  paralysis  of,  accompanied  by  irritation 

of  sympathetic  nerve,  95 
Lipaemia  in  trench  nephritis,  163 

retinalis  in  diabetes,  167,  168 
Lumbar     anaesthesia,     indirect     paralysis     of 

cranial  nerves  following,  43 
puncture  in  papilloedema,  23 
vertebrae,  green  deposits  in  periosteum  of, 

in  chloroma,  74 
Lymphoma  of  orbit,  73 

Macula,    cherry-red    spot    at,    in    amaurotic 

family  idiocy,  217,  218 
escape  of,  in  vascular  lesions,  7 
star  figure  at,  in  chlorosis,  75 


292 


INDEX 


Macula,  tortuosity  of  small  vessels  in  region 

of,  In  retinal  arterio-sclerosis,  54,  55 
Marular  representation,  6 
Malaria,  retinal  haemorrhage  in,  284 
Male  fern.     See  Aspidium  (Filix  max). 
Meninges,  tubercles  in  choroid  without  invasion 

of,  in  miliary  tuberculosis,  199,  200,  201 
Meningitis,  basal  syphilitic,  causing  paralysis 

of  ocular  nerves,  195 

cerebro-spinal,  amaurosis  without  ophthal- 

moscopic  signs  in  children  with,  203 

intra-ocular  metastatic  infections  in, 

202 
ocular  lesions  in,  201—204 

palsies  in,  203,  204 
papillcedema  in,  202 
post-basic,    complicated    by   panophthal- 

mitis,  204 
retention  of  pupillary  light  reflex  in, 

40 
tuberculous,  cedema  of  disc  in,  201 

section    through    choroidal    tubercle 

from  case  of,  200 
tubercles  in  choroid  in,  198,  199 
Metastatic  infections,  intra-ocular,  in  cerebro- 
spinal  meningitis,  202 

Migraine  and  epilepsy,  supposed  affinity  be- 
tween, 239 

associated  ocular  complications  of,  237 
frequent  attacks  of,  followed  by  complete 

ocular  paralyses,  240 
hemianopic,  241 
ophthalmoplegic,  239 
temporary      hemianopia      accompanying 
course  of  dividing  line  between  seeing 
and  blind  halves,  6 
Mikulicz's  disease,  247 

enlargement  of  glands  of  head  in,  247 
Miner's  nystagmus.     See  Nystagmus,  miner's. 
Miosis,  following  removal  of  portion  of  sympa- 
thetic nerve,  96 
Mcebius'  sign  in  chronic  nephritis,  139 

in  exophthalmic  goitre,  173 
Movement,   appreciation  of,   investigation   of 

visual  field  for,  after  gunshot  injuries,  11 
Muscles,    ocular,     defective    movements     in 

exophthalmic  goitre,  177,  178 
paralysis  of,  causes,  104 

congenital  and  hereditary,  109 
following  diphtheria,  212 
in  disseminate  sclerosis,  125 
in  syringomelia,  128 
Myasthenia  gravis,  129 

accompanied  by  intermittent  diplopia,  105 
age  and  sex  incidence,  129 
and  nystagmus,  32 
ocular  signs  of,  130 
prognosis,  130 
symptoms,  129,  130 

Mydriasis,  in  irritation  of  sympathetic  nerve,  96 
Myelitis,  acute, 

loss  of  sight  first  symptom  of,  121 
•with  retro-bulbar  neuritis,  120 
Myelocytes,    infiltration    of   tissues    with,    in 

chloroma,  74 

Myotonia  atrophica,  cataract  in,  130 
characteristic  features  of,  130 

Naphthalin,    administration    of,    causing    in- 
crease of  salts  in  aqueous  humour,  277 
experimental,    in    large    doses    producing 

double  cataract,  277 
Nebulae,  corneal,  nystagmus  due  to,  28 
Nephritis,  acute,  incidence  of  retinitis  in,  132 
chronic,  ocular  signs  in,  139 
different  forms  of,  incidence  of  retinitis  in, 

132 

interstitial,  chronic  retinitis  in,  133 
papillcedema  in,  149,  150 
parenchymatous,     and      arterio-sclerotic 
retinitis,  ophthalmoscopic  appear- 
ances compared,  155,  156 
chronic  retinitis  infrequent  in,  133 
See  alto  Trench  nephritis. 


Nerve  fibre  layer  of  retina,  hemorrhages  into 

34,  35 

fibres  controlling  dilator  pupillse,  94,  95 
medullated,  in  retina,  231 

ophthalmoscopic       appearances, 

232 

relative  scotoma  in,  231 
varicose,   development    in    leuka?mic 

retinitis,  73 

in  renal  retinitis,  148,  149 
Nervous  system,  central,  changes  in  amaurotic 

family  idiocy,  218,  219 
diseases  of  accompanied  by  nystagmus,  31 
Neuritis,  alcoholic,  nystagmus  and,  32 
optic,  12 

alcoholic,  263,  264 

and  choked  disc,  distinction  between, 

17 

in  diseases  of  pituitary  body,  186 
in  encephalitis  lethargica,  112 
in  lead  poisoning,  272 
mild,  in  disseminate  sclerosis,  123 
rarity    in    destruction    of    brain    by 

softening  or  clot,  19 
secondary,    interstitial,    in    cerebro- 
spinal  meningitis,  203 
retro-bulbar,  256 

and  acute  myelitis,  association  of,  120 
complete  loss  of  sight  in,  256 
dilatation  of  pupil  in,  100 
hemianopia  in,  100 
in  diabetes,  170 
in  exophthalmic  goitre,  179 
symptoms,  256 
toxic,  causes  of,  262 
Neuro-retinitis,  in  lead  poisoning,  272 
New  York  Neurological  Society,  Commission  of 
inquiry  into  eyestrain  in  refation  to  nervous 
disturbances,  103 
Night  blindness,  222 
acquired,  223 
associated  with  xerosis  of  conjunctiva,  223 

treatment,  223 
congenital,  222 

genealogical  tree  of,  222 
in  hereditary  diseases  of  retina,  222 
in  syphilitic  chorio-retinitis,  223 
Nitro-benzol  poisoning,  276 

dark  colour  of  fundus  in,  276 
ocular  disturbances  in,  276 
symptoms,  276 
Novarseno-benzol,  administration  of,  followed 

by  changes  in  optic  nerve,  280 
Nucleus,  sixth,  paralysis  of,  appearances  in,  106 
Nyctalopia  in  leukaemic  retinitis,  73 

See  also  Night  blindness. 
Nystagmoid  movements  of  eyes,  23 
Nystagmus,  23 

albinism  and,  26,  27 

causes  of,  26 

circumductory,  percentage,  24 

diseases  of  nervous  system  accompanied 

by,  31 

disjunctive,  percentage,  24 
due  to  corneal  nebulae,  28 
head  nodding  and  (spasmiis  nutans),  33 
hereditary,  26,  27 
horizontal,  percentage,  24 
in  disseminate  sclerosis,  125 
in  encephalitis  lethargica,  113 
in  Friedreieh's  ataxia,  120 
in  lead  poisoning,  272 
in  syringomyelia,  129 
irregular,  percentage,  24 
labyrinthine,  32 

'  in  disease  of  internal   ear  involving 

semi-circular  canals,  32 
mode  of  production  in  normal  indi- 
viduals, 32,  33 

production    in    cases    of    middle-ear 
diseases  with  flstulous  communica- 
tion between  middle  and  internal 
ears,  33 
provocation,  and  disappearance  of  33 


INDEX 


293 


Nystagmus,  miner's,  29 

causation,  29,  30 

diplopia  in,  31 

eye  movements  in,  24,  31 

refractive  errors  and,  31 
mixed,  percentage,  24 
occupational,  29 

See  aluo  Nystagmus,  miner's, 
percentage  of  cases  of  posterior  uveitis 

developing,  28 
rotatory,  percentage,  24 
symptoms,  25 
true,  24 
unilateral,  25 

vertical,  25 
variations,  26 
vertical,  percentage,  24 
visual  acuity  in,  26 
voluntary,  27 

Occipital  cortex  and  optic  radiations,  gunshot 
injury  to  inferior  hemianopia  following, 
perimeter  charts  showing,  8,  9 
poles,  injury  to,  scotoma  following,  6 
Ochronosis  of  sclerotic,  277 

in  alkaptonuria,  277 
Ocular  nerves,  involvement  in  cavernous  sinus 

thrombosis,  91 
paralysis   of,  due  to   basal  syphilitic 

meningitis,  195 
due  to  involvement  in    gummatous 

disease  of  orbit,  195 
in  cerebral  syphilis,  frequency,  196 
uncertain  value  as  localising  sign  in 

cerebral  disease,  105 
tension  in  diabetes,  172 
(Edema,  retinal,  in  renal  retinitis,  139 
Ophthalmological  Society,  Committee  of  Inves- 
tigation on  Bisulphide  of  Carbon  Poisoning, 
275 

Ophthalmoplegia  externa,  104 
chronic  progressive,  109 
unilateral,  110 
syphilitic,  196 
tetanic,  210,  211 
with  inco-ordination  of  lower  limbs,  cause 

of,  108 
Optic  atrophy  and  ataxia,  partial  dissociation, 

118 

in  disseminate  sclerosis,  122,  123 
following    poisoning    by    aspidium 

(F(7ir  ma«),  279 

in  diseases  of  pituitary  body,  185 
hereditary,  214 

See  also  Leber's  disease, 
in  diabetics,  171 

in  general  paralysis  and  tabes,  116, 1 17 
in  one  eye,  with  papillcedema  in  other, 

produced  by  cerebral  tumour,  21 
in  lead  poisoning,  272 
in  oxycephaly,  234,  235 

due  to  antecedent  papillcedema, 

235 

pathogenesis  of,  236 
in  retinal  arterio-sclerosis,  67 
in  syringomyelia,  129 
in  wood  alcohol  blindness,  265,  266 
parasyphilitic,  195 
primary,     production    by    pituitary 

tumours,  16 

resulting  in  hydrocephalus,  111 
relation  to  ataxia,  117 
retention  of  pupillary  light  reflex  in, 

41 

syphilitic,  195 
foramen,    tumour    pressure    at,    causing 

obstruction  of  ophthalmic  artery,  16 
nerve,  anatomical  relations  of,  46 

changes  in,  following  administration 

of  novarseno-benzol,  280 
degeneration    of,    following    use    of 

atoxyl,  279 

division  resulting  from  fracture  in- 
volving anterior  cranial  fossa,  48 


Optic   nerve  head,  changes  in,  in  diseases  of 

pituitary  body,  186 
location  of  bi-nasal  hemianopia  in,  40 
sheath,  haemorrhage  into,  45,  48 

accompanied   by  retinal  haemor- 
rhage, 47 

following  fracture  of  skull,  46,  47 
signs  of,  47 

radiations  and  occipital  cortex,  gunshot  in- 
jury to  inferior  hemianopia  follow- 
ing, perimeter  charts  showing,  8,  9 
injury  to,  producing  defects  of  visual 

fields,  94 
lesions  of,  associated  with  quadrantic 

hemianopia,  10 
Orbit,  gummatous  disease  of,  paralysis  of  ocular 

nerves  due  to  involvement  in,  195 
leukaemic  infiltrations  of,  73 
lymphoma  of,  73 

Orbits,  bleeding  into,  in  infantile  scurvy,  187 
Otitis  media  causing  cavernous  sinus'  throm- 
bosis, 92 

Oxycephaly,  cause  of  unknown,  234 
eye  symptoms  in,  235 
exoplithalmos  and  optic  atrophy  in,  234 
morbid  anatomy,  234 
pathology,  234 
sex  incidence,  234 
Oyster  shucker's  keratitis,  256 


Palpebral  fissure,  widening  of,  in  irritation  of 

sympathetic  nerve,  98 
Palsies,  ocular,   in  cerebro-spinal   mjningitis, 

203,  204 

rarity  in  congenital  syphilis,  196 
syphilitic,  195,  196 
Palsy,  ocular,  in  migraine,  239 
Panophthalmitis,  complicating  pneumonia,  205 
post-basic  meningitis,  204 
pyaemia  and  septicaemia,  204 
following  embolism  in  retinal  artery,  207 
Papilla,  changes  in,  associated  with  cerebral 

syphilis,  195 
whiteness  of  temporal  half,  in  alcoholic 

amblyopia,  263 
Papillcedema 

accompanying  cerebral  tumour,  17 

appearances  of  disc  in,  17 

causes  of,  18 

cerebral  syphilis  with,  19 

changes  in  vessels  in,  14 

contraction  of  field  of  vision  in,  22 

developmsnt  of,  when  prevented,  16 

displacement  of  retina  in,  13 

fan-shaped  figure  in  retina  in,  147 

haemorrhages  in,  15 

homolaterality  of,  20 

in  cerebral  tumour,  149,  150 

in  cerebro-spinal  meningitis,  202 

in  chlorosis,  75 

in  oxycephaly  resulting  in  optic  atrophy, 

235 
in  renal  disease,  149 

prognostic  significance,  150 
in  syringomyelia,  129 
incidence,  18 

varying  with  site  of  cerebral  tumour, 

18 

increase  of  intra-cranial  pressure  in,  15,  16 
ipso-laterality  and  contra-laterality  com- 
pared in  cases  of  cerebral  tumour,  20 
lumbar  puncture  in,  23 
onset  of,  long  preceded  by  cerebral  tumour, 

19,  20 

ophthalmoscopic  appearances  in,  17 
pathogenesis  of,  15 
pathology,  13 
premonitory  signs  of,  17 
preservation  of  sight  by  cerebral  decom- 
pression operations  in,  21,  22 
production  in  one  eye  only,  16,  23 

and  optic  atrophy  in  other  produced 
by  cerebral  tumour,  21 


294 


INDEX 


rapilloedema,  rarity  in  cerebral  or  intra-cranial 

haemorrhage,  1!) 
in  cerebral  softening,  19 
in  encephalitis  lethargica,  112 
recurrence  of,  19 

cerebral  tumours  without,  19 
relation  of  ophthalmoscopie  appearances 

to  histological  changes  in,  14,  15 
relative    immunity   from    in   tumours    of 

cortical  area,  18 
sclerotic  changes  in,  14 
severity  in  tumours  of  cerebellum,  18 
slight  degrees  of,  17 
white  dots  radiating  from  yellow  spot  in, 

17 

with  cerebral  abscess,  21 
1'apillo-macular  bundle,  changes  in    alcoholic 

optic  neuritis  limited  to,  264 
course  of,  264 
degeneration    in    subjects    of    tobacco- 

amblyopia,  261 

Paralysis,  general,  cycloplegia  in,  116 
intra-ocular  lesions  in,  114 
optic  atrophy  in,  116 
indirect,  of  cranial  nerves,  41,  42 

following  spinal  anaesthesia,  42 
ocular,  frequently  first  symptom  of  ence- 
phalitis lethargica,  113 
permanent    and    complete    resulting 
from    continuous    attacks    of    mi- 
graine, 240 
recurrent   temporary    in    association 

with  severe  headache,  240 
of  external  muscles  of  eyes,  causes  of,  104 
of  limbs,   accompanied   by   irritation   of 

sympathetic  nerve,  95 
of  muscles  supplied  by  third  nerves  in 
association  with    weakness    of    lower 
limbs,  107 

of  ocular   muscles,  congenital  and  here- 
ditary, 109 

following  diphtheria,  212 
in  disseminate  sclerosis,  125 
in  syringomelia,  128 
of  ocular  nerves,  syphilitic,  195,  196 

uncertain   value    as    localising    sign 

in  cerebral  disease,  105 
of  pupil  in  association  with  loss  of  upward 

movement,  109 
of  sixth  nerve,  accompanied  by  monocular 

diplopia,  44,  45 
bilateral,  complete,  106 
bilateral,  incomplete,  106 
of  sympathetic  nerve,  95 
of  third  nerve,  sometimes  "  distant  "  in 

causation,  107 
of  upward  movement  of  eyes  in  association 

with  paralysis  of  levators,  108 
Paresis  of  third  nerve  in  association  with  cyclic 

contraction  and  dilatation  of  pupil,  228 
Pedigrees  of  blue  sclerotics,  224 
Perivasculitis  in  lead  poisoning,  272 
Phenol,    solution    of,    antiseptic    application 
followed  by  temporary  total  blindness,  276 
Photophobia  in  migraine,  237 
Physiological  block  causing  defects  in  visual 

fields,  183,  184 
Pigment  epithelium  changes  in  diabetic  iritis, 

167 

Pipe-stem  sheathing  of  retinal  artery,  59 
Pituitary  body,  diseases  of,  complicated  by  de- 
fects of  visual  fields,  accompanied 
by  acromegaly,  185 
factors  concerned  in,  179 
ocular  lesions  complicating,  179 
ophthalmoscopic  changes  in,  185,  186 
disturbances   of,    in   relation   to   Leber's 

disease,  216,  217 
tumours   of,    operation   on,  followed   by 

restoration  of  visual  fields,  184 
production  of  primary  optic  atrophy 

by,  16 

Pneumonia,  complicated  by  panophthalmitis, 
205 


Polio-encephalitis,  ocular  symptoms  associated 

with,  114 

Pons,  tegmentum  of,  paralysis  of,  107 
Pregnancy,  disturbances  during,  without  renal 

retinitis,  138 

nephritis  of,  retinitis  associated  with,  136 
See  also  Retinitis,  renal,  of  pregnancy. 
Pregnant    woman,    restriction    of    temporal 

visual  fields  in,  184 

Proptosis  accompanying  thrombosis  of  cavern- 
ous sinus,  91 

in  exophthalmic  goitre,  174 
in  chloroma,  74 
in  infantile  scurvy,  187 
unilateral,   in  healthy   new-born   infants, 

188 

Pseudo-glioma,  204 
Pseudo-nystagmus,  23 
Pseudo-papiltedema  in  chlorosis,  75 
Ptosis  in  encephalitis  lethargica,  112 
in  jaw  blinking,  227 
in  myasthenia  gravis,  130 
Puerperal    amaurosis,   retention  of    pupillary 

light  reflex  in,  40 

Pupil  abnormalities,  in  disseminate  sclerosis  12f> 
changes  in,  in  syringomyelia,  129 
condition,    in    paralysis    of    sympathetic 

nerve,  95 

cyclic  contraction  and  dilatation  of,  asso- 
ciated with  third  nerve  paresis,  228 
paralysis  of,  in  association  with  paralysis 

of  downward  movement,  109 
reaction,  hemianopic,  39 
Wernicke's,  37 

sluggish,  in  retinal  arterio-sclerosis  55 
with  return  of  vision,  39 
Pupils,  dilatation  of,  following  administration 

of  aethylhydrocuprein  (optochin),  230 
in  epilepsy,  102 

Purpura,  retinal  haemorrhages  in,  77 
Pyaemia,  ocular  complications,  204 


Quinine  as  an  abortifacient,  267 
blindness,  267 

changes  in  retina  in,  269 
degrees  of,  267,  268 
experimental  production,  269 
peripheral  constriction  of  visual  fields 

in,  268 
symptoms,  267,  263,  269 

Raynaud's  disease,  retinal  haemorrhages  in,  78 

spasm  of  retinal  vessels  in,  78 
Recti,  external,  paralysis  of,   following  diph- 
theria, 212 
Rectus,  external,  paralysis  of,  106 

involvement  in  polio-encephalitis,  114 
Refraction,  alterations  of,  in  diabetes,  169 
errors  of,  and  miner's  nystagmus,  31 

trivial  importance  of,  in  aetiology  of 

epilepsy  or  chorea,  104 
resulting  'in  epilepsy,  question  of,  103 
Reptiles,    development    of    superior    corpora 

quadrigemina  in,  41 
Retina  and  choroid  of  one  eye,  dissemination 

of  chloroma  into,  75 
aneurisms  of   large  arteries  and  veins  of, 

230 

anterior  part,  infection  in  leprosy,  213 
appearances  at  death,  48,  49 
blood  vessels  of,  abnormalities,  230 

engorgement     and      distension      in 

leukaemia,  70,  71 
in  cyanosis  retinae,  83 
mode  of  protection  against  increase  of. 
intra-cranial    pressure    in    case    of 
tumour  pressure  at  optic  foramen, 
16 

spasm  in  Raynaud's  disease,  73 
tortuosity  of,  230 

branches    of    artery    in,    constriction    of 
lumen,  in  arterio-sclerosis,  54 


INDEX 


295 


Retina,  central  blood  vessels  of,  course  back- 
wards from  retina,  16 
changes  in,  in  quinine  blindness,  269 
degeneration  of,  following  use  of  atoxyl, 

279 
detachment   of,  following  death  of  cysti- 

cercus  within  eye,  282 
in  renal  retinitis,  150 

of  pregnancy,  138 
displacement  in  papilloedema,  13 
fan-shaped  figure  in,  in  papilloedema,  147 
first,   second,   and   third   changes   in,   in 

leukaemia,  70,  71,  72 

formation  of  fat  in,  in  renal  retinitis,  142 
ganglion  cells  of,   changes  in  amaurotic 

family  idiocy,  219,  220 
medullate  nerve  fibres  in,  231 
nerve  fibre    layer  of,  haemorrhages  into, 

34,  35 
oedema  of,  accompanying  cavernous  sinus 

thrombosis,  91 
pupillomotor  and  visual  functions,  relation 

between,  38 
section    through,    from    case    of   arterio- 

sclerotic  retinitis,  58 

star  figure  in,  in  renal  retinitis,  142 — 148 
stimulation   by  strong  light  to   produce 

contraction  of  pupil,  38 
upper  parts  of,  representation  in  posterior 

calcarine  fissure,  8,  9 
various  portions  of,  quarters  of  visual  area 

in  which  represented,  11,  12 
vein  riding  over  thickened  artery  in,  52 
venous  engorgement  in,  in  epilepsy,  102 
thrombosis,  84 

with  haemorrhages,  in  erythraemia,  82 
white  patches  in,  in  renal  retinitis,  140 — 

143 
white  spots  or  dots  in,  in  arterio-sclerotic 

retinitis,  55,  56,  57,  58 
zones  of,  connection  of  zones  of  visual  area 

with,  7,  8 

See  also  Cyanosis  retinae. 
See  also  Haemorrhages,  retinal. 
Retinae,  ophthalmoscopic  appearances  in  case 

of  haematemesis,  80 
Retinitis,  arterio-sclerotic,  55 

and  renal,  differences  between,  55 

tabulated,  58 

and  retinitis  associated  with  parenchy- 
matous  nephritis,  ophthalmoscopic 
appearances  compared,  155,  156 
evolution  from  retinal  arterio-sclerosis, 

57 

ophthalmoscopic  appearances,  56 
prognosis,  62 
unilateral,  57 
diabetes  as  cause  of,  161 
diabetic,  age  incidence,  165 

differentiation  as  separate  entity,  161 
heightened  blood  pressure  in,  161,  163 

absence  of,  163 
prognosis,  164,  165 
in  chronic  interstitial  nephritis,  133 
incidence  in  acute  nephritis,  132 

in  different  forms  of  nephritis,  132 
in  trench  nephritis,  132,  133 
infrequent    in    chronic    parenchymatous 

nephritis,  133 
in  lead  poisoning,  272 
leukaemic,  72 

ophthalmoscopic  appearances,  72,  73 
white  areas  seen  in,  73 
narrowing  of  visual  fields  and  nyctalopia 

in,  73 

pigmentosa,  night  blindness  in,  222 
punctata  albescens,  night  blindness  in,  222 
renal,  131 

and  diabetic,  differences  between,  164 
case  of  subsidence  of  without  abate- 
ment of  renal  disease,  135,  136 
differing  types  of,  154,  155 
in  uraemia,  133,  134 
increase  of  blood  pressure  in,  136 


Retinitis,  renal,  of  pregnancy,  136 

non-recurrence  in  successive  preg- 
nancies, 137 
prognosis,  136 
recovery  of  vision  in,  137 
retinal  detachments  in,  151 
severity  of,  138 
statistics  of,  137 

ophthalmoscopic  changes  in,  139,  140 
or  arterio-sclerotic,  time  required  for 
disappearance  of  different  types  of 
retinal  exudate  in,  diagram  show- 
ing, 157 
prognosis,  63 

prognostic  significance,  135 
recurrence,  protection  against,  158 
retinal  detachments  in,  150 
sex  incidence,  134 
statistics  of,  131,  132 
subjective  symptoms,  152 
subsidence  of,  152 
unilateral,  157,  153 
varicose  nerve  fibres  in,  14S,  149 
vascular  changes  in,  143 
syphilitic,  194 

Rhythmic    movements    of    body    favouring 
development  of  miner's  nystagmus,  31 


Santonin,    ingestion    of,  followed    by    yellow 

vision,  278 
Sclerosis,  disseminate,  changes  in  visual  fields 

in,  126 

disc  changes  in,  123,  124 
loss  of  sight  in    124 
"  mild  optic  neuritis  "  in,  123 
nystagmus  in,  125 
ocular    lesions    first    symptoms    of, 

122 

ophthalmoscopic  changes  in,  122 
optic  atrophy  in,  122,  123 
pathological  changes  in,  127,  123 
pupillary  abnormalities  in,  126 
retro-bulbar  neuritis  in,  124,  125 
insular,    accompanied     by     intermittent 

diplopia,  105 

Sclerotic  changes  in  papilloedema,  14 
ochronosis  of,  377 

in  alkaptonuria,  277 
principal  seat  of  changes  due  to  leprosy 

infection,  213 
Scotoma,    central,    in    Leber's    disease,    215, 

216 

in  tabes,  119 
or    paracentral    in    thrombosis     of 

retinal  veins,  86,  87 
following  injury  to  occipital  poles,  6 
homonymous,   narrow,   with  softening  of 
brain  at   bottom   of   calcarine   fissure, 
position  of,  10 

in  tpbacco-amblyopia,  258,  260 
position  in  obstruction   of  right  posterior 

cerebral  artery,  6,  7 
in  relation  to  fixation  point,  6,  7 
Scotomata    in    visual    fields    accompanying 

disease  of  pituitary  body,  184 
Scurvy,    infantile    (Barlow's    disease),  ocular 

manifestations  of,  187 
Septicaemia,  ocular  complications  of,  204 
Silver,  nitrate  of,  effect  on  conjunction,  274 
Sixth    nerve,    involvement,     in    encephalitis 

lethargica,  112 
nucleus,  changes  in  encephalitis  lethargica, 

114 
paralysis   of,   accompanied  by  monocular 

diplopia,  44,  45 
in  cerebral  syphilis,  196 
indirect,  41,  42 

Skull  bones,  greenish  thickening  in  chloroma,  74 
fracture  of,  followed  by  haemorrhage  into 

optic  nerve  sheath,  46,  47 
Sleeping  sickness,  use  of  atoxyl  in,  followed  by 

blindness,  279 
Smoking,  toxic  effects  in  diabetics,  171 


296 


INDEX 


Snow  blindness,  251 
symptoms,  251 
Soap  placed  in  eyes  resulting  in  conjunctivitis, 

100 
Sodium  salicylate,  large  doses  of, 

causing  temporary  loss  of  sight,  270 

resulting  in  complete  blindness,  270 

Spasm  of  retinal  arteries  complicating  retinal 

arterio-sclerosis,  64,  65 
Spasmus    nutans.    See    Head    nodding    and 

nystagmus. 

Sphincter  pupillae,  not  inhibited  by  sympa- 
thetic nerve,  96 
Spinal  anaesthesia,  indirect  paralysis  of  cranial 

nerves  following,  42,  43 
Sporotrichosis  of  conjunctiva,  285 
Squint,    paralytic,    internal,    associated    with 

polio-encephalitis,  114 
Star  figure  in  retina, 

accompanying  small  white  kidney,  156 
in  renaj  retinitis,  142,  143,  144—148 
Stellwag's  sign  in  chronic  nephritis,  139 

exophthalmic  goitre,  173 
Stereoscopic  vision,  loss  of,  in  disturbances  of 

visual  orientation,  93 

Strabismus   following   post-diphtheritic   para- 
lysis of  extrinsic  ocular  muscles,  212 
Stria  of  Gennari,  1,  2,  3 
Subarachnoid  space,  cerebral,  further  passage 

of  blood  entering,  46 

of  vaginal  sheath  of  optic  nerve,  relation 
of  increase  in  intracranial  pressure  in 
papillcedema  to,  15,  16 
Sulcus  calcarinus,  1 
cunei,  1,  2 
lingualis,  1,  2 
limitans, 

inferior  areas  striatae,  1,  2 
superior  areas  striatse,  1,  2 
Sun  blindness.    See  Eclipse  blindness. 
Sympathetic  nerve,  irritation  of,  accompany- 
ing paralysis  of  the  limbs,  95 

symptoms,  96 
lesions  of,  94 

paralysis  of,  condition  of  iris  in,  96 
condition  of  pupil  in,  95 
test  for,  95,  96 
removal  of  portion  of,  followed  by  miosis, 

96 

sphincter  pupillae  not  inhibited  by,  96 
Syphilis,  acquired,  rarely  followed  by  interstitial 

keratitis,  191,  192 
cerebral,  changes  in  papilla  associated  with, 

195 
with  papiltedema,  19 

trephining    contra-indicated    in, 

23 

paralysis  of  ocular  nerves  in,  196 
congenital,  followed   by  interstitial  kera- 
titis, 191 

rarity  of  ocular  palsies  in,  196 
ocular  manifestations  of,  190 
Syringomyelia,  nystagmus  in,  129 
ocular  changes  in,  128 
ophthalmoscopic  changes  in,  129 
paralysis  of  ocular  muscles  in,  123 
pupillary  changes  in,  129 


Tabes  dorsalis,  bi-temporal  hemianopia  in,  119 
central  scotoma  in,  119 
complicated  by  bi-nasal  hemianopia 

40 

cycloplegia  in,  116 
impairment  of  colour  vision  in,  120 
intra-ocular  lesions  in,  114,  115 
optic  atrophy  in,  116 
Tay    Sachs    disease.     See    Amaurotic    family    ' 

idiocy. 

Tetanus  following  eye  injuries,  210,  211  ' 
Third  nerve,  affection  of,  in  diseases  of  pituitarv 

body,  187 

connected   with    cerebral    blood    vessels,    i 
43 


Third  paralysis,  aetiology  discussed,  241 
in  cerebral  syphilis,  196 
bilateral, 

with  crossed  hemiplpgia,  196 
without  hemiplegia,  1!>(5 
unilateral,    without    hemiplegia 

196 

indirect,  41,  43 
of  muscles  supplied  by,  in  association 

with  weakness  of  lower  limbs,  107 
sometimes    "  distant  "    in    causation, 

107 

paresis  of,  in  association  with  cyclic  con- 
traction and  dilatation  of  pupil,  228 
Thrombosis,   cerebral,   associated   with   retinal 

arterio-sclerosis,  59,  60 
of  cavernous  sinus,  90 
causes  of,  90 
changes  in  eye  due  to  development  of 

91 

of  central  retinal   vein,   glaucoma  super- 
vening in,  68 

of  cranial  sinuses  in  erythrsemia,  82 
of  retinal  artery,  66 

and    veins,    complicating   facial    ery- 
sipelas, 207 

followed  by  blindness,  66 
of  retinal  vein,  central,  development 

of  glaucoma  in,  87 
of  retinal  veins, 

age  and  sex  incidence,  85,  90 

albiiminuria  in,  85,  86 

blood-pressure  in,  85 

causation,  85 

development    of    tortuous    veins    on 

disc  after  recovery  from,  87,  83 
loss  of  vision  in,  87 
ophthalmoscopic  appearances,  86 
prognosis,  88,  89 
retinal  haemorrhages  in,  86 
unilateral,  89 
visual  fields  in,  86,  87 
of  right  posterior  cerebral  artery,   visual 
fields  in  case  of,  diagrams  showing,  6,  7 
Tobacco  amblyopia,  258 
age  incidence,  259 
degeneration  of  papillo-macular  bundle  In, 

261 

kind  of  smoking  principally  causing,  259 
main  features  of,  258 
means  of  curing,  259 
pallor  of  disc  in,  260 
pathology  of,  theories  as  to,  261 
scotoma  in,  258,  260 
visual  fields  in,  260 
Tobacco-plant,  immunity  to  effects  of,  upon 

ingestion  by  animals,  260 
Torticollis,  ocular,  225 
treatment,  227 
usually  congenital,  226 
resulting  from  contraction  of  sterno-cleido- 
mastoid,  and  ocular  torticollis,  differen- 
tiation between,  226 
Trench  nephritis, 

incidence  of  retinitis  in,  132,  133 
increase  of  blood-pressure  in  cases  with 

retinitis,  136 
lipeemia  in,  163 
Trephining  in  choked  disc,  22 
in  papillcedema,  22 

centra-indications  for,  22,  23 
Trypanosomiasis,  eye  symptoms  in,  283 
Tuberculosis,  miliary,  development  of  tubercles 

in  choroid  in,  198 
Tuberculous  disease  of  eye,  197 

rarity,  197 
gross,  of  iris  and  ciliary  body,  197 

Uraemia,  blindness  of,   retention  of    pupillary 

light  reflex  in,  40 

onset  of,  accompanied  by  loss  of  vision,  1  ">3 
renal  retinitis  in,  133,  134 

associated  with  exophthalmic  goitre 
139 


INDEX 


297 


Tvcitis.  posterior,  percentage  of  eases  develop- 
ing nystagmus,  23 

Vaquez's  disease.    Xcc  Erythrsemia. 
Vascular  changes  in  renal  retinitis,  149 

lesions,  escape  of  macula  in,  explained,  7 
Vitsculitis  in  lead  poisoning,  272 
Vein,  retinal,  central, 

development  of  glaucoma  in,  87 
thrombosis  of,  glaucoma  supervening  on, 

68 
Veins,  retinal,  engorgement,  with  haemorrhages 

in  erythraemia,  82 
thrombosis  of,  84 
tortuous,  accompanying  sinus  thrombosis, 

91 
Vision,  central,  position   of  cortex  connected 

with,  6 

cortical  representation,  1 
defects  of,  in  fields  of  both  eyes,  congruous 

and  superimposable,  12 
in  hydrocephalus,  cause,  111 
disturbances  of,  during  pregnancy,  parturi- 
tion or  puerperium,  138 
eclampsia  accompanied  by,  138 
loss  of,  following  prolonged  lactation,  248 
in  bisulphide  of  carbon  poisoning,  275 
in  disseminated  sclerosis,  124 
in  lead  poisoning,  272 
in  recurrent  intra-ocular  haemorrhage, 

244 

in  thrombosis  of  retinal  veins,  87 
with  slight  ophthalmoscopic  changes 

in  erythraemia,  80,  81 
macular  or  "central,  centre  for,  12 
permanent  injury  to,  in  herpes  ophthal- 

micus,  246 
preservation  in  papillcedema  by  cerebral 

decompression  operations,  21,  22 
recovery  of,  after  gunshot  wounds  of  brain, 

10,  11 

after  lead  poisoning,  272,  273 
in  Leber's  disease,  215 
in  renal  retinitis  of  pregnancy,  137 
relation  of  superior  corpora  quadrigemina 

to,  41 

return  of,  pupil  reaction  with,  39 
stages  at  which  affected  in  case  of  suicide 

by  charcoal  fumes,  49 
subserved  by  periphery  of  retina,  centre 

for,  12 
Visual  acuity  in  nystagmus,  26 

area,  regions  of  in  which   various  parts 

of  retina  are  represented,  11,  12 
zones  of,   connection  with  zones  of 

retina,  7,  8 
phenomena,   subjective,   following  injury 

to  visual  cortex,  11 
field,  contraction  in  papillcedema,  22 

investigation  for  appreciation  of  move- 
ment, after  gunshot  injuries,  11 
periphery,    defects    at,    situation    of 
lesions"  producing,  10 


Visual  field?,  affected  by  wounds  of  upper  lip  of 

posterior  calcarine  fissure,  8,  9 
changes  in,  disseminate  sclerosis,  126 
contraction  of,  in  hysteria,  99 
in  leukaemic  retinitis,  73 
correlation    of    gunshot    wounds    of 

brain  with,  4,  5 
defects   in,   complicating  diseases   of 

pituitary  body,  180 
accompanied  by  acromegaly, 

185 
due  to  physiological  block, 

183,  184 
due  to  injury  to  optic  radiations, 

94 

irregular,    complicating   diseases 
of   pituitary   body,    180,    181, 
182,  183 
distortion  of,  accompanying  disease  of 

pituitary  body,  184,  185 
in  case  of  thrombosis  of  right  posterior 

cerebral  artery,  6,  7 
in  tabes,  central  scotoma  in,  119 
in  thrombosis  of  retinal  veins,  86,  87 
in  tobacco-amblyopia,  260 
losses  for  colour,  11 
peripheral    constriction    in     quinine 

blindness,  268 
scotomata  in,  accompanying  disease 

of  pituitary  body,  184 
temporal,     restriction     in    pregnant 

women,  184 
orientation,  disturbances  of,  93 

accompanied  by  loss  of  stereoscopic 

vision,  93 
Visual  orientation  accompanying  brain  lesions, 

93 

principal  manifestations,  93 
site  of  cerebral  lesion  in,  94 
Vitamin,    fat-soluble,    absence     of,    in     diet 
causing  kerato-malacia,  188, 189 

Wernicke's  pupil  reaction,  37 
White  patches  in  retina,  in  renal  retinitis,  140 
l^g 

spots  or  dots  in  retina  in  arterio-sclerotic 

retinitis,  55,  56,  57,  58 
Women,  rarity  of  gonococcal  iritis  in,  208 
Wood  alcohol  blindness,  265,  266 

optic  atrophy  in,  265,  266 
poisoning,  265 

by  inhalation,  266 
deaths  from,  265,  266 
Word  blindness,  congenital,  220 
prognosis,  221 
sex  incidence  and  frequency,  221,  222 

Yellow  spot  region,  haemorrhage  in,  35 

white  dots  radiating  from,  in  papilloe- 

dema,  17 

vision   following   administration    of   san- 
tonin, 278 


INDEX    OF   AUTHORS 


Abercrombie,  44 

Bull,  134,  159,  205 

Dock,  172,  173,  174 

Abrahams,  24 

Bullard,  129 

Dodd,  120 

Adam,  138,  225 

Bumke,  115 

Dransart,  24,  31 

Adams,  60,  63 

Bunge,  264,  265 

Duane,  25 

Adamiik,  143,  148 

Burrows,  224 

Dufour,  '!'>- 

Aircv,  239 

Burton,  277 

Dunn,  73,  112 

Albert,  211 

Buzzard,  20,  95,  118,  129 

Dupuy-Dutemps,  47,  82,  99 

Albrecht,  277 

Byers,  209 

Duverger,  16 

Allbutt,  Clifford,  120 

Dwight,  92 

Anderson,  H.  K.,  96 

Calderaro,  213 

Anderson,  J.,  45,  163 

Cameron,  260 

Eales,  242,  243 

Arndt,  116 

Campbell,  129,  246,  247 

Eddowes,  224 

Atkinson,  251 

Carlini,  78 

Edmunds,  18,  40,  73,  171,  263 

Aubineau,  32 

Carlyll,  219 

Elschnig,  132 

Axenfeld,  97 

Carpenter,  182,  192 

Ely,  112 

Ayer,  112 

Chaillous,  285 

Englebach,  82 

Chapoto,  44 

Enslin,  235 

Bailey,  23 

Charcot,  98,  126  239 

Balacescn,  177 

Chevallereau,  78,  79 

Fabian,  74 

Ballaban,  75,  281 

Chisholm,  121 

Falta,  175 

Ballet,  178 

Christian,  82 

Farabelli,  64 

Bannister,  75 

Clarke,  189,  279 

Fawcett,  26 

Barabaschew,  268 

Coats,  51,  53,  54,  57,  74,  84,  87, 

Fearnsides,  130,  195 

Barker,  139 

88,  90,  219,  220,  229,  267 

Ferrier,  93,  103 

Barlow,  200,  202,  250 

Coborn,  83 

Fildes,  195 

Barre,  16 

Cockayne,  224,  225,  227 

Fisch,  97 

Barrett,  260 

Coggin,  207 

Fisher,  J.   H.,    184,  190,  192, 

Bastian,  221 

Cohen,  219 

216,  217,  221,  230,  240.  261 

Batten,  F.,  Ill,  114,  130,  202, 

Collier,  42 

Flechsig,  232 

203 

Collins,  E.  T.,  190,  191,  213,  252 

Fleischer,  12.". 

Batten,  R.,  78,  215 

Collins,  \V.,  196 

Flemming,  205,  232 

Beaumont,  235 

Conlon,  224 

Fletcher,  Morlev,  234,  235,  236 

Beck,  279 

Cooper,  B.,  170 

Folker,  272,  273 

Becker,  167 

Connor,  258,  262 

Forster,  118 

Bedell,  285 

Cosse,  209,  210 

Forti,  184 

Beevor,  40 

Coupland,  91 

Foster,  Lanckton,  258 

Belt,  135 

Courtois-Sufflt,  210 

Fraenkel,  283 

Benson,  229,  230 

Crawford,  188 

Franceschi,  278 

Bernstein,  74 

Critchett,  274 

Franke,  230 

Berry,  109,  262 

Cuignet,  225 

Fridenberg,  32,  265,  266 

Best,  38 

Cunningham,  243 

Friedenwald,  81,  82,  236 

Bickerton,  103 

Cushing,  Harvev,   39,   40,   41, 

Fries,  79 

Bielschowskv,  44,  122 

42,  75,   111,   179,   181,   183, 

Frith,  74 

Birch-Hirsctifeld,  261,  266,279 

184,  185,  186,  187 

Fromaget,  210 

Bistis,  96 

Frost,  215,  262 

Bitsch,  75 
Blaauw,  78 

Daland,  251 
Dalen,  261 

Fuchs,  40,  119,  21;>,  2;...,  :2»i2 

Bordlev,  75 

Dallwig,  227 

Galezowski,  98,  119,  159,  272 

Borel,  98 

Daniels,  283 

Gallemaerts,  75 

Borland,  188 

Darling,  168 

Garrod,  A.  E.,  161,  170,  277 

Borthen,  212 

Dawson,  127 

Gatti,  270 

Bouchard,  277 

De  Lapersonne,  181,  227 

Gay,  249 

Boudier,  202 

Delord,  209,  210 

Gayet,  177 

Bouveret,  44 

Delpech,  275 

Geis,  61 

Bowman,  245 

Derby,  G.,  248 

Germain,  92 

Bradbourne,  109 

De  Schweinitz,  19,  22,  23,  41, 

Gescherdt,  283 

Brailev,  44 

42,  55,  67,  69,  70,  95,   182, 

Ghosh,  68 

Bramwell,  B.,  122 

184,  258,  260,  261,  262,  264, 

Gibb,  130 

Bramwell,  E.,  95,  110,  125,  129 

266,  268,  269,  270,  271,  272, 

Gibson,  271 

Brav,  234 

275,  278,  279,  283 

Giesen,  97 

Bristowe,  19,  175,  178 

Deutschman,  2,~>:; 

Ginsberg,  143,  143 

Brown,  82 

De  Vries,  83 

Giroux,  210 

Bruce,  108 

De  Wecker,  78 

Goldzieher,  107 

Brunner,  217 

Dewev,  268 

Gonin,  47,  254 

Buchnan,  22:> 

Dixoii,  219 

Goodhart,  176 

INDEX  OF  AUTHORS 


299 


Gordon.  11  -J 

Jackson,  Hughlings,  19,  64,  201 

Maxwell,  209 

Gould,  16!) 

James,  Autson,  156,  170 

Mayou,  96,  97 

Goulden,  120,  174 

Jcaffreson,  270 

Mavweg,  244 

Gowrrs.    18,    20.    21.    7."..    102, 

Jeanselme,  212,  213 

McColIum,  189 

106,  103,  111,  ll.->,  116,  117, 

Jessop,  96,  174 

McCullough,  285 

160,  241,  27:! 

Juler,  44,  163 

McGillivrav,  283 

Graefe,  27 

Junius,  116 

Mclntosh,  195 

Graham.  G.,  170,  209,  210 

McKee,  202 

Green,  237 

Kako,  163,  165,  167,  170,  171 

McMullen,  109 

Greenfield,  130 

Kamocki,  167 

Melland,  112 

Greenwood,  64 

Kampherstein,  125,  126 

Meller,  73 

<;  reeves,  114.  230 

Kapuscinski,  481 

Mendel,  117,  266 

Griffith,  172.  173.  174,  282,  283 

Karplus,  94,  208,  241 

Mierzejewski,  106 

Grimsdale,  169 

Katavama,  279 

Mikuliez,  247 

Grout,  78 

Katz,"  121 

Milev,  132,  134,  135 

Gruening,  265,  269 

Kelly,  266 

Miller,  82 

Gubl.T,  241 

Kendal,  260 

Mitra,  68 

Gulliver,  44 

Ker,  210 

Mohr,  20,  274 

Gunii.  4.->.  :>o.  :>7.  60,  63,  98, 

Kerschbaumer,  73 

Monk,  94 

100,  12:>,  227,  230,  262 

Kiep,  209 

Moore,  98 

Kingdon,  217 

Morgenwroth,  280 

Habershon,  214 

Kingborn,  75 

Morrax,  209,  212,  213,  285- 

H  addon,  33 

Kipp,  284 

Morton,  262 

Hall   74,  112 

Hancoi-k,  33,  21<> 

Knapp,  40,  207,  240,  241 
Kneiss,  78 

Mott,  219,  220 
Mules,  70 

Hant-s.  139 

Knies,  157,  278 

Miiller,  286 

Harlan,  41,  98,  100,  109,  256, 

Konigstein,  83 

Murray,  172 

267 

Koster,  279 

Harinan,  28,  192,  224,228 

Kreidel,  94 

"Vatral     ft't 

Harris,  6,  24,  115.  239,  241 
Head,  195,  246,  247 
Heagey,  112 

Hobb   74 

Kubert,  212 
Kuinmol.  247 
Kurt,  117 

->.1Ut  I,  O-> 

Iv'aumhotf,  84 
Netter,  112 
Nettleship,  19,  27,  41,  45,  63, 

Heed,'  40 
Heimann.  25 
Heine,  167,  172,  202 
Heitz,  115,  116 

Lafon,  30 
Lagrange,  73 
Landolt.  227 
Lang,  B.  T.,  231 

109,  117,  119,  123,  133,  134, 
135,  137,  154,  157,  159,  163, 
164,  165,  196,  215,  221,  222, 
230,  235,  248,  249,  250,  256, 
;)60   *)6"?   ^63 

Henderson,  96 
Henschen,  10,  18 
Hepburn,  223 
Herrfordt,  209 

Lang,  W.,  40,  178 
Langdon,  65,  97 
Langley,  96,  97 
Lauber,  143,  148 

Xeustatter,  25 
Xieden,  30,  31,  227,  276 
Xorris,  214,  215,  271 

Herringham,  132,  134,  138,  159 

Lawford,    18,    122,    171,    192, 

Noyes,  64 
Nuel   148 

Hertel,  187 

196,  201,  215,  252,  283 

Hess,  38 

Lawson,  283 

Hesse,  38,  39,  97 

Leber,  73,  171,  207,  214,  222, 

Ohm,  31 

Heyl,  167 

254 

Okamoto,  279 

Higgens,  176 

Ledingham,  74 

Oliver,  249,  270,  272 

Hill,  16 

Lees,  250 

Opin,  148 

Hine,  11,  109 

Lehndortf,  74 

Oppenheim,  116 

Hinshelwood,  220,  221 

Lenoble,  32 

Ord,  44,  45 

Hippel,  22,  83,  110,  230 

Levinsohn,  110 

Orloff,  286 

Hirschberg,  75,  163 

Levy,  280 

Ormerod,  240 

Hirst,  137 

Lewis,  B.,  116 

Onnond,  26,  242,  24'J 

Hoffmann,  79,  167 

Liebrecht,  47 

Osier,  81,  82 

Holden,  114,  122,  269 

Liebreich,  154 

Holder,  46 

Lister,  W.  T.,  3,  7,  8 

Hotlowav,  22,  184 

Litten,  75,  143 

Pagenstecher,  75 

Holmes,  G.,  3,  7,  8,  9,  10,  11, 

Liveing,  237 

Panas,  254 

13,  14,  20,  93,  94,  109,  120, 

Llewellyn,  29,  31 

Parisotti,  64 

122,  218,  219,  242 

Lloyd,  95,  239,  240 

Parker,  82 

Hopkins,  Gowland,  188 

Lobel,  119 

Parkinson,  John,  80,  82,  280 

Horrax,  93 

Lodato,  97 

Parsons,  12,  13,  14,  96,  218, 

Horsley,  20,  118 

Loewe,  272 

219,  261,  285 

Hotz,  277 

Lucas,  81,  82 

Paton,  13,  14,  18,  20,  124 

Howe,  114 

Lundsgaard,  169 

Patry,  177 

Hubbell,  239 

Lutembacher,  82 

Pean,  177 

Hudson,  73,  74 

Pechin,  67 

Hulke,  148 

Mackav,  252,  253 

Peter,  197 

Hurst,  99 

M.u  kenzie,  69,  163,  230 

Peters,  277 

Hutchinson,  82,  97,  159,  160, 

Mackinlay,  276 

Pfeiffer,  74 

191,  192,  193,  196,  244,  245, 

Maddox,  24 

Posey,  75,  82,  137 

258,  273 

Maloney,  118 

Pothner,  112 

Mannheim,  172 

Poynton,  218,  219 

Inouve,  87 

Manson,  284 

Price,  40 

Ishihara,  22:; 

Marples,  201 

Pringle,  178 

Marx,  125 

Pringle,  J.  A.,  229,  230 

Jaboulav,  177 

Maude,  178 

Pusey,  75 

Jackson",  £.,  82,  92 

Ma  was,  143 

Putman,  91 

300 


Randolph,  201,  202,  204,  25»i 

Soelberg-  Wells,  25 

Valuilc.  1274 

Ransome,  70 

Sorger,  72 

Van  der  Brugh,  226 

Reber,  42,  55,  171 

Spicer,    Holme*.   24,    28,   188, 

Van  Millingen,  2.~>!) 

Reid,  30 

189,  240 

Vaquez,  80 

Reinhard,  67 

Spiller,  23,  95,  123 

Veasey,  40 

R*rabe,  282 

Spillman,  159 

Verhaeghe,  192 

Riddoch,  11,  93 

Spratling,  102 

Verhoeff,  219,  221 

Risely,  169,  227 

Standish,  41 

Villard,  248 

Robertson,  Argyll,  284 

Starr,  103,  128 

Vinsonneau,  202 

Rochon-Dtivigneaud,  115,  116, 

Stephenson,  114,  138,  188,  181), 

Voss,  178 

141,  143,  148 

224,  226,  227 

Vossius,  265 

Rockliffo,  163,  213,  283 

Stevens,  103 

Rollin,  67 

Still,  111 

Rose,  278 

Stock,  73 

Wadsworth,  91,  227 

Rosenbach,  106 

Stood,  271,  272 

Wagenmann,  65,  2:;2 

Rosenhauch,  286 

Strzeminski,  187 

Walker,  39,  40,  179,  180,  181 

Russel,  Risien,  31,  216,  217 

Stuelp,  279 

183,  185,  186 

Sweet,  79 

Warner,  178,  201 

Sachs,  64  112,  217 

Symns,  99 

Watson,  82 

Saenger,  78,  109,  173 

Weber,  P.,  80 

Samuelsohn,  263 

Tay,  Waren,  217 

Weeks,  98,  242,  283 

Sargent,  70 

Taylor,  James,  19,  31,  32,  66 

Wegeforth,  112 

Sattler,    173,   211,    237,    242, 

Taylor,  Johnson,  215 

Weisberg,  129 

244,  246 

Terrien,  202 

Weiss,  241 

Saxer,  129 

Terson,  192 

Wells,  196 

Schafer,  7,  93 

Thomas,   129,   221,  222,   241, 

Werner,  73,  282 

Schieck,  148 

285 

West,  132,  175,  178 

Sohlesinger,  128,  129 

Thompson,  24,  44 

Westcott,  75 

Schmeisser,  201 

Thomsen,  104,  241 

White,  Hale,  16!) 

Schmidt-Rirapler,  172 

Thomson,  19 

White,  276 

Schobl,  282 

Thursfleld,  203,  248 

Wigglesworth,  103 

Schweigger,  167 

Tilney,  114 

Wilbrand,  10,  78,  109,  173 

Scott,  32,  33 

Tobias,  117 

Wilde,  196 

Sedgwick,  215 

Tooke,  199 

Wilder,  285 

Semple,  137,  141,  148 

Tredgold,  127,  128 

Williams,  27,  207 

Sharkey,  19,  122,  123,  172 

Treitel,  223 

Wilmer,  243,  265 

Shears,  262 

Tucker,  112 

Wilson,  Kinneir,  112 

Shiplev,  288 

Turk,  73 

Wilson,  Read,  195 

Silcock,  176,  178 

Tyson,  266 

Wood,  Casey,  99 

Simon,  152 

Wood,  D.  J.,  230,282 

Simonds,  189 

Ulrich,  79 

\Voods,  Hiram,  242,  244,  283 

Sinclair,  110,  222,  227 

Uhthotf,  17,  18,  19,  20,  21,  22, 

Worms,  210 

Slinger,  118 

31,  106,  110,  119,  122,  123, 

Smith,  Elliot,  1 

124,  125,  126,  128,  181,  186, 

Smith,  Priestley,  47,  66,  68, 

187,  195,  196,  235,  236,  242, 

Zentmaver,  217 

259,  274 
Snell,  27,  28,  30,  270,  276 

262,  263,  264 
Usher,  48,  49,  233 

Ziegler,  247,  265 
Ziehe,  97 

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